Cystinuria: An inherited disorder due to defective reabsorption of CYSTINE and other BASIC AMINO ACIDS by the PROXIMAL RENAL TUBULES. This form of aminoaciduria is characterized by the abnormally high urinary levels of cystine; LYSINE; ARGININE; and ORNITHINE. Mutations involve the amino acid transport protein gene SLC3A1.Cystine: A covalently linked dimeric nonessential amino acid formed by the oxidation of CYSTEINE. Two molecules of cysteine are joined together by a disulfide bridge to form cystine.Amino Acid Transport Systems, Basic: Amino acid transporter systems capable of transporting basic amino acids (AMINO ACIDS, BASIC).Amino Acid Transport System y+Large Neutral Amino Acid-Transporter 1: A CD98 antigen light chain that when heterodimerized with CD98 antigen heavy chain (ANTIGENS, CD98 HEAVY CHAIN) forms a protein that mediates sodium-independent L-type amino acid transport.Amino Acid Transport Systems: Cellular proteins and protein complexes that transport amino acids across biological membranes.Biological Transport: The movement of materials (including biochemical substances and drugs) through a biological system at the cellular level. The transport can be across cell membranes and epithelial layers. It also can occur within intracellular compartments and extracellular compartments.Amino Acid Transport System L: A sodium-independent neutral amino acid transporter system with specificity for large amino acids. One of the functions of the transporter system is to supply large neutral amino acids to the brain.Amino Acids: Organic compounds that generally contain an amino (-NH2) and a carboxyl (-COOH) group. Twenty alpha-amino acids are the subunits which are polymerized to form proteins.Amino Acid Transport Systems, Neutral: Amino acid transporter systems capable of transporting neutral amino acids (AMINO ACIDS, NEUTRAL).Antigens, CD98 Heavy Chain: A transmembrane glycoprotein subunit that can dimerize with a variety of light chain subunits (ANTIGENS, CD98 LIGHT CHAINS). This protein subunit serves a diverse array of functions including amino acid transport and cell fusion. Its function is altered depending which of the light chain subunits it interacts with.Antigens, CD98: A heterodimeric protein that is a cell surface antigen associated with lymphocyte activation. The initial characterization of this protein revealed one identifiable heavy chain (ANTIGENS, CD98 HEAVY CHAIN) and an indeterminate smaller light chain. It is now known that a variety of light chain subunits (ANTIGENS, CD98 LIGHT CHAINS) can dimerize with the heavy chain. Depending upon its light chain composition a diverse array of functions can be found for this protein. Functions include: type L amino acid transport, type y+L amino acid transport and regulation of cellular fusion.Antigens, CD98 Light Chains: A family of light chains that bind to the CD98 heavy chain (ANTIGENS, CD98 HEAVY CHAIN) to form a heterodimer. They convey functional specificity to the protein.Amino Acids, Neutral: Amino acids with uncharged R groups or side chains.Sinorhizobium: A genus of gram-negative, aerobic, nonsporeforming rods which usually contain granules of poly-beta-hydroxybutyrate. (From Bergey's Manual of Determinative Bacteriology, 9th ed)Structure-Activity Relationship: The relationship between the chemical structure of a compound and its biological or pharmacological activity. Compounds are often classed together because they have structural characteristics in common including shape, size, stereochemical arrangement, and distribution of functional groups.Photochemotherapy: Therapy using oral or topical photosensitizing agents with subsequent exposure to light.Aminolevulinic Acid: A compound produced from succinyl-CoA and GLYCINE as an intermediate in heme synthesis. It is used as a PHOTOCHEMOTHERAPY for actinic KERATOSIS.Photosensitizing Agents: Drugs that are pharmacologically inactive but when exposed to ultraviolet radiation or sunlight are converted to their active metabolite to produce a beneficial reaction affecting the diseased tissue. These compounds can be administered topically or systemically and have been used therapeutically to treat psoriasis and various types of neoplasms.Heme: The color-furnishing portion of hemoglobin. It is found free in tissues and as the prosthetic group in many hemeproteins.Fluorine: A nonmetallic, diatomic gas that is a trace element and member of the halogen family. It is used in dentistry as flouride (FLUORIDES) to prevent dental caries.Dihydroxyphenylalanine: A beta-hydroxylated derivative of phenylalanine. The D-form of dihydroxyphenylalanine has less physiologic activity than the L-form and is commonly used experimentally to determine whether the pharmacological effects of LEVODOPA are stereospecific.Sensitivity and Specificity: Binary classification measures to assess test results. Sensitivity or recall rate is the proportion of true positives. Specificity is the probability of correctly determining the absence of a condition. (From Last, Dictionary of Epidemiology, 2d ed)Magnetic Resonance Imaging: Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques.Fluorine Compounds: Inorganic compounds that contain fluorine as an integral part of the molecule.Nucleosides: Purine or pyrimidine bases attached to a ribose or deoxyribose. (From King & Stansfield, A Dictionary of Genetics, 4th ed)Isotope Labeling: Techniques for labeling a substance with a stable or radioactive isotope. It is not used for articles involving labeled substances unless the methods of labeling are substantively discussed. Tracers that may be labeled include chemical substances, cells, or microorganisms.Subcellular Fractions: Components of a cell produced by various separation techniques which, though they disrupt the delicate anatomy of a cell, preserve the structure and physiology of its functioning constituents for biochemical and ultrastructural analysis. (From Alberts et al., Molecular Biology of the Cell, 2d ed, p163)Isotopes: Atomic species differing in mass number but having the same atomic number. (Grant & Hackh's Chemical Dictionary, 5th ed)Deoxyglucose: 2-Deoxy-D-arabino-hexose. An antimetabolite of glucose with antiviral activity.NorbornanesKinetics: The rate dynamics in chemical or physical systems.LLC-PK1 Cells: Epithelial cell line originally derived from porcine kidneys. It is used for pharmacologic and metabolic studies.Biological Transport, Active: The movement of materials across cell membranes and epithelial layers against an electrochemical gradient, requiring the expenditure of metabolic energy.Delayed-Action Preparations: Dosage forms of a drug that act over a period of time by controlled-release processes or technology.Tablets: Solid dosage forms, of varying weight, size, and shape, which may be molded or compressed, and which contain a medicinal substance in pure or diluted form. (Dorland, 28th ed)Tablets, Enteric-Coated: Tablets coated with material that delays release of the medication until after they leave the stomach. (Dorland, 28th ed)Chemistry, Pharmaceutical: Chemistry dealing with the composition and preparation of agents having PHARMACOLOGIC ACTIONS or diagnostic use.Biological Availability: The extent to which the active ingredient of a drug dosage form becomes available at the site of drug action or in a biological medium believed to reflect accessibility to a site of action.Methylcellulose: Methylester of cellulose. Methylcellulose is used as an emulsifying and suspending agent in cosmetics, pharmaceutics and the chemical industry. It is used therapeutically as a bulk laxative.Drug Compounding: The preparation, mixing, and assembling of a drug. (From Remington, The Science and Practice of Pharmacy, 19th ed, p1814)Excipients: Usually inert substances added to a prescription in order to provide suitable consistency to the dosage form. These include binders, matrix, base or diluent in pills, tablets, creams, salves, etc.Administration, Oral: The giving of drugs, chemicals, or other substances by mouth.Technology, Pharmaceutical: The application of scientific knowledge or technology to pharmacy and the pharmaceutical industry. It includes methods, techniques, and instrumentation in the manufacture, preparation, compounding, dispensing, packaging, and storing of drugs and other preparations used in diagnostic and determinative procedures, and in the treatment of patients.Amino Acids, Cyclic: A class of amino acids characterized by a closed ring structure.Amino Acid Sequence: The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.Molecular Sequence Data: Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.Trigger Points: Discrete spots in taut bands of muscle that produce local and referred pain when muscle bands are compressed.Nostoc: A form-genus of CYANOBACTERIA in the order Nostocales. Trichomes composed of spherical or ovoid vegetative cells along with heterocysts and akinetes. The species form symbiotic associations with a wide range of eukaryotes.Amino Acids, DiaminoNitrogen Fixation: The process in certain BACTERIA; FUNGI; and CYANOBACTERIA converting free atmospheric NITROGEN to biologically usable forms of nitrogen, such as AMMONIA; NITRATES; and amino compounds.AcetyleneCyanobacteria: A phylum of oxygenic photosynthetic bacteria comprised of unicellular to multicellular bacteria possessing CHLOROPHYLL a and carrying out oxygenic PHOTOSYNTHESIS. Cyanobacteria are the only known organisms capable of fixing both CARBON DIOXIDE (in the presence of light) and NITROGEN. Cell morphology can include nitrogen-fixing heterocysts and/or resting cells called akinetes. Formerly called blue-green algae, cyanobacteria were traditionally treated as ALGAE.Nostoc commune: A form species of spore-producing CYANOBACTERIA, in the family Nostocaceae, order Nostocales. It is an important source of fixed NITROGEN in nutrient-depleted soils. When wet, it appears as a jelly-like mass.Nitrogenase: An enzyme system that catalyzes the fixing of nitrogen in soil bacteria and blue-green algae (CYANOBACTERIA). EC 1.18.6.1.Anabaena: A genus of CYANOBACTERIA consisting of trichomes that are untapered with conspicuous constrictions at cross-walls. A firm individual sheath is absent, but a soft covering is often present. Many species are known worldwide as major components of freshwater PLANKTON and also of many saline lakes. The species ANABAENA FLOS-AQUAE is responsible for acute poisonings of various animals.GlycogenMicrocystins: Cyclic heptapeptides found in MICROCYSTIS and other CYANOBACTERIA. Hepatotoxic and carcinogenic effects have been noted. They are sometimes called cyanotoxins, which should not be confused with chemicals containing a cyano group (CN) which are toxic.Positron-Emission Tomography: An imaging technique using compounds labelled with short-lived positron-emitting radionuclides (such as carbon-11, nitrogen-13, oxygen-15 and fluorine-18) to measure cell metabolism. It has been useful in study of soft tissues such as CANCER; CARDIOVASCULAR SYSTEM; and brain. SINGLE-PHOTON EMISSION-COMPUTED TOMOGRAPHY is closely related to positron emission tomography, but uses isotopes with longer half-lives and resolution is lower.Fluorine Radioisotopes: Unstable isotopes of fluorine that decay or disintegrate emitting radiation. F atoms with atomic weights 17, 18, and 20-22 are radioactive fluorine isotopes.Tropanes: N-methyl-8-azabicyclo[3.2.1]octanes best known for the ones found in PLANTS.Radiopharmaceuticals: Compounds that are used in medicine as sources of radiation for radiotherapy and for diagnostic purposes. They have numerous uses in research and industry. (Martindale, The Extra Pharmacopoeia, 30th ed, p1161)NortropanesFluorodeoxyglucose F18: The compound is given by intravenous injection to do POSITRON-EMISSION TOMOGRAPHY for the assessment of cerebral and myocardial glucose metabolism in various physiological or pathological states including stroke and myocardial ischemia. It is also employed for the detection of malignant tumors including those of the brain, liver, and thyroid gland. (From Martindale, The Extra Pharmacopoeia, 30th ed, p1162)Tissue Distribution: Accumulation of a drug or chemical substance in various organs (including those not relevant to its pharmacologic or therapeutic action). This distribution depends on the blood flow or perfusion rate of the organ, the ability of the drug to penetrate organ membranes, tissue specificity, protein binding. The distribution is usually expressed as tissue to plasma ratios.Scalp: The outer covering of the calvaria. It is composed of several layers: SKIN; subcutaneous connective tissue; the occipitofrontal muscle which includes the tendinous galea aponeurotica; loose connective tissue; and the pericranium (the PERIOSTEUM of the SKULL).Tomography, Emission-Computed: Tomography using radioactive emissions from injected RADIONUCLIDES and computer ALGORITHMS to reconstruct an image.Research Support, U.S. Gov't, Non-P.H.S.Research Support, U.S. Gov't, P.H.S.Research Support, Non-U.S. Gov'tMetabolomics: The systematic identification and quantitation of all the metabolic products of a cell, tissue, organ, or organism under varying conditions. The METABOLOME of a cell or organism is a dynamic collection of metabolites which represent its net response to current conditions.Research Support, U.S. GovernmentResearch Support, American Recovery and Reinvestment ActResearch Support, N.I.H., ExtramuralResearch Support, N.I.H., IntramuralResearch Support as Topic: Financial support of research activities.PubMed: A bibliographic database that includes MEDLINE as its primary subset. It is produced by the National Center for Biotechnology Information (NCBI), part of the NATIONAL LIBRARY OF MEDICINE. PubMed, which is searchable through NLM's Web site, also includes access to additional citations to selected life sciences journals not in MEDLINE, and links to other resources such as the full-text of articles at participating publishers' Web sites, NCBI's molecular biology databases, and PubMed Central.Cholestasis, Intrahepatic: Impairment of bile flow due to injury to the HEPATOCYTES; BILE CANALICULI; or the intrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC).Cholestasis: Impairment of bile flow due to obstruction in small bile ducts (INTRAHEPATIC CHOLESTASIS) or obstruction in large bile ducts (EXTRAHEPATIC CHOLESTASIS).Liver Diseases: Pathological processes of the LIVER.Liver Cirrhosis, Biliary: FIBROSIS of the hepatic parenchyma due to obstruction of BILE flow (CHOLESTASIS) in the intrahepatic or extrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC; BILE DUCTS, EXTRAHEPATIC). Primary biliary cirrhosis involves the destruction of small intra-hepatic bile ducts and bile secretion. Secondary biliary cirrhosis is produced by prolonged obstruction of large intrahepatic or extrahepatic bile ducts from a variety of causes.TextilesGastroenterology: A subspecialty of internal medicine concerned with the study of the physiology and diseases of the digestive system and related structures (esophagus, liver, gallbladder, and pancreas).Cholangitis, Sclerosing: Chronic inflammatory disease of the BILIARY TRACT. It is characterized by fibrosis and hardening of the intrahepatic and extrahepatic biliary ductal systems leading to bile duct strictures, CHOLESTASIS, and eventual BILIARY CIRRHOSIS.Liver Transplantation: The transference of a part of or an entire liver from one human or animal to another.Ursodeoxycholic Acid: An epimer of chenodeoxycholic acid. It is a mammalian bile acid found first in the bear and is apparently either a precursor or a product of chenodeoxycholate. Its administration changes the composition of bile and may dissolve gallstones. It is used as a cholagogue and choleretic.Cholagogues and Choleretics: Gastrointestinal agents that stimulate the flow of bile into the duodenum (cholagogues) or stimulate the production of bile by the liver (choleretic).

*Aminolevulinic acid

... increases the mitochondrial activity thereby helping in activation of respiratory system Krebs Cycle and Electron Transport ... δ-Aminolevulinic acid (also dALA, δ-ALA, 5ALA or 5-aminolevulinic acid), an endogenous non-protein amino acid, is the first ... The US FDA approved aminolevulinic acid hydrochloride (ALA HCL) for this use in 2017. Aminolevulinic acid is being studied for ... In plants, algae, bacteria (except for the α-proteobacteria group) and archaea, it is produced from glutamic acid via glutamyl- ...

*SLC1A1

... amino-acid transporter 3 is a member of the high-affinity glutamate transporters which plays an essential role in transporting ... "Entrez Gene: SLC1A1 solute carrier family 1 (neuronal/epithelial high affinity glutamate transporter, system Xag), member 1". ... SLC1A1, also known as excitatory amino-acid transporter 3 (EAAT3), is a protein that in humans is encoded by the SLC1A1 gene. ... Excitatory amino acid transporter Glutamate transporter Solute carrier family GRCh38: Ensembl release 89: ENSG00000106688 - ...

*AAAP family

"Coupled and uncoupled proton movement by amino acid transport system N". EMBO Journal. 20 (24): 7041-51. doi:10.1093/emboj/ ... Since Systems A are electrogenic which Systems N are not, the amino acid:cation stoichiometries may differ. Fischer, WN; Loo, ... "Low and high affinity amino acid H+-cotransporters for cellular import of neutral and charged amino acids". Plant Journal. 29 ( ... Six AAAPs in A. thaliana are well characterized and transport neutral and charged amino acids with varying specificities and ...

*SLC7A11

... is a member of a heterodimeric Na+-independent anionic amino acid transport system highly specific for cystine and ... the amino acid transport activity system xc-". Pflugers Archiv. 442 (2): 286-96. doi:10.1007/s004240100537. PMID 11417227. ... the light chain of amino acid transport system xc-". Antioxidants & Redox Signaling. 2 (4): 665-71. doi:10.1089/ars.2000.2.4- ... "Entrez Gene: SLC7A11 solute carrier family 7, (cationic amino acid transporter, y+ system) member 11". Lutgen V, Resch J, ...

*SLC6A19

"Molecular cloning of mouse amino acid transport system B0, a neutral amino acid transporter related to Hartnup disorder". J. ... SLC6A19 is a system B(0) transporter that mediates epithelial resorption of neutral amino acids across the apical membrane in ... Solute carrier family 6 member 19 also known as the sodium-dependent neutral amino acid transporter B(0)AT1 or system B(0) ... Bröer S (January 2008). "Amino acid transport across mammalian intestinal and renal epithelia". Physiol. Rev. 88 (1): 249-286. ...

*SLC38A2

Jones HN, Ashworth CJ, Page KR, McArdle HJ (2006). "Cortisol stimulates system A amino acid transport and SNAT2 expression in a ... "Transcriptional control of the human sodium-coupled neutral amino acid transporter system A gene by amino acid availability is ... 2006). "Amino acid starvation induces the SNAT2 neutral amino acid transporter by a mechanism that involves eukaryotic ... 2006). "Characterization of the amino acid response element within the human sodium-coupled neutral amino acid transporter 2 ( ...

*SLC1A5

Kudo Y, Boyd CA (August 2002). "Changes in expression and function of syncytin and its receptor, amino acid transport system B( ... Neutral amino acid transporter B(0) is a protein that in humans is encoded by the SLC1A5 gene. Glutamate transporter Solute ... "Entrez Gene: SLC1A5 solute carrier family 1 (neutral amino acid transporter), member 5". Tailor CS, Nouri A, Zhao Y, Takeuchi Y ... Uchiyama T, Matsuda Y, Wada M, Takahashi S, Fujita T (April 2005). "Functional regulation of Na+-dependent neutral amino acid ...

*GcvB RNA

... encodes a small non-coding RNA involved in the regulation of a number of amino acid transport systems as well as amino acid ... GcvB RNA also is involved in regulating a variety of genes involved in amino acid biosynthesis such as ilvC, gdhA, thrL and ... "The gcvB gene encodes a small untranslated RNA involved in expression of the dipeptide and oligopeptide transport systems in ... "The gcvB gene encodes a small untranslated RNA involved in expression of the dipeptide and oligopeptide transport systems in ...

*Gamma-Amino-beta-hydroxybutyric acid

... and l-γ-Amino-β-Hydroxybutyric Acids in GABA Receptor and Transport Test Systems" (pdf). Journal of Neuroscience. 1 (2): 132- ... γ-Amino-β-hydroxybutyric acid (GABOB), also known as β-hydroxy-γ-aminobutyric acid (β-hydroxy-GABA), and sold under the brand ... Effect of gamma-amino-beta-hydroxybutyric acid (GABHB) on experimentally-induced epileptic activity]" [Effect of γ-amino-β- ... García-Flores E, Farías R (1997). "γ-Amino-β-hydroxybutyric acid as add-on therapy in adult patients with severe focal epilepsy ...

*SLC3A2

Amino Acid Transport System L at the US National Library of Medicine Medical Subject Headings (MeSH) This article incorporates ... "Amino acid transport of y+L-type by heterodimers of 4F2hc/CD98 and members of the glycoprotein-associated amino acid ... "Entrez Gene: SLC3A2 solute carrier family 3 (activators of dibasic and neutral amino acid transport), member 2". Kucharzik T, ... Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y (2004). "CATs and HATs: the SLC7 family of amino acid transporters ...

*List of MeSH codes (D12.776.157)

... amino acid transport systems, acidic MeSH D12.776.157.530.200.249.500 -- amino acid transport system x-ag MeSH D12.776.157.530. ... amino acid transport system a MeSH D12.776.157.530.200.500.200 -- amino acid transport system asc MeSH D12.776.157.530.200.500. ... amino acid transport systems, basic MeSH D12.776.157.530.200.374.600 -- amino acid transport system y+ MeSH D12.776.157.530. ... cationic amino acid transporter 2 MeSH D12.776.157.530.200.374.750 -- amino acid transport system y+l MeSH D12.776.157.530. ...

*Photorhabdus luminescens

... encodes a small non-coding RNA involved in the regulation of a number of amino acid transport systems as well as amino acid ...

*CD98

Amino Acid Transport System L at the US National Library of Medicine Medical Subject Headings (MeSH). ... Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y (2004). "CATs and HATs: the SLC7 family of amino acid transporters ... CD98 is a glycoprotein that is a heterodimer composed of SLC3A2 and SLC7A5 that forms the large neutral amino acid transporter ... Boado RJ, Li JY, Nagaya M, Zhang C, Pardridge WM (1999). "Selective expression of the large neutral amino acid transporter at ...

*Expanded genetic code

... but often the various transport systems can handle unnatural amino acids with apolar side-chains. In the second case, a ... while the added amino acids are called non-standard amino acids (NSAAs), or unnatural amino acids (uAAs; term not used in ... or non-canonical amino acids. The first element of the system is the amino acid that is added to the genetic code of a certain ... An amino acid auxotrophic expression host is supplemented with an amino acid analog during target protein expression. This ...

*Serotonin-norepinephrine reuptake inhibitor

Serotonin is synthesized from an amino acid called L-tryptophan. Active transport system regulates the uptake of tryptophan ... The same motif can be found in reboxetine where it is constrained in a morpholine ring system. Some studies have been made ... 150 mg/day), it acts on serotonergic and noradrenergic systems, whereas at high doses (>300 mg/day), it also affects ... Although the perception and transmission of pain stimuli in the central nervous system have not been fully elucidated, ...

*Membrane transport protein

This transport system normally removes cysteine from the fluid destined to become urine and returns this essential amino acid ... such as glucose or amino acids. When the lipid bilayer is impermeable to the molecule needing transport, active transport is ... Excitatory amino acid transporters (EAATs) EAAT1 EAAT2 EAAT3 EAAT4 EAAT5 Glucose transporter Monoamine transporters, including ... Unlike channel proteins which only transport substances through membranes passively, carrier proteins can transport ions and ...

*Blood-brain barrier

... as well as the selective transport of molecules such as glucose and amino acids that are crucial to neural function. ... "Lipoic acid affects cellular migration into the central nervous system and stabilizes blood-brain barrier integrity". J. ... No difference in brain uptake of glucose, amino acids, organic acids, purines, nucleosides, or choline was observed between ... Other methods used to get through the BBB may entail the use of endogenous transport systems, including carrier-mediated ...

*Phytotoxin

The plants are slowly starved of these amino acids and eventually DNA synthesis stops. ESPS Inhibitors affect grasses and ... They are medically important by interfering with components of the nervous system affecting membrane transport, protein ... ALS Inhibitors affect grasses and dicots by inhibiting the first step in some amino acid synthesis, acetolactate synthesis. ... Alkaloids are derived from amino acids, and contain nitrogen. ...

*Serotonin

... because the transport system which brings tryptophan across the blood-brain barrier is also selective for the other amino acids ... High plasma levels of other large neutral amino acids compete for transport and prevent the elevated plasma tryptophan from ... In animals including humans, serotonin is synthesized from the amino acid L-tryptophan by a short metabolic pathway consisting ... aromatic amino acid decarboxylase (DDC) and the coenzyme pyridoxal phosphate. The TPH-mediated reaction is the rate-limiting ...

*Enterocyte

Peptide and amino acid uptake. Peptidases in the glycocalyx cleave proteins to amino acids or small peptides. Enteropeptidase ( ... Galactose uses the same transport system. Fructose, on the other hand, crosses the apical membrane of the enterocyte, using ... Smaller lipids are transported into intestinal capillaries, while larger lipids are processed by the Golgi and smooth ... Microvilli on the apical surface increase surface area for the digestion and transport of molecules from the intestinal lumen. ...

*Angomonas deanei

The bacteria synthesise amino acids, vitamins, and haem for the protozoan. In return the protozoan offers its enzymes for the ... The bacteria are known to provide essential nutrients to the host, and provide electron transport system for the production of ... the genomic cooperation between bacterium and host in the synthesis of essential amino acids is heavily influenced by multiple ... complete metabolic pathways for the biosysnthesis of amino acids, lipids and nucleotides, that are absent in the bacterium. ...

*Iminoglycinuria

... part of the system of Na-K-Cl cotransporters) couple with the amino or imino acids on the molecular level and transport them ... instead of the amino group found in amino acids. Proline is considered and usually referred to as an amino acid, but unlike ... The neutral amino acid transporter SLC6A19 (affecting glycine, proline, and other neutral amino acids like cysteine and ... "Neutral amino acid transport mediated by ortholog of imino acid transporter SIT1/SLC6A20 in opossum kidney cells" (Free full ...

*Metallothionein

Cysteine is a sulfur-containing amino acid, hence the name "-thionein". However, the participation of inorganic sulfide and ... Metallothioneins likely participate in the uptake, transport, and regulation of zinc in biological systems. Mammalian MT binds ... In this way the thionein-metallothionein becomes a key component of the zinc signaling system in cells. This system is ... of its constituent amino acid residues. MT was discovered in 1957 by Vallee and Margoshe from purification of a Cd-binding ...

*KPNA3

... encodes a protein similar to certain nuclear transport proteins of Xenopus and human. The predicted amino acid sequence shows ... The similarities among these proteins suggests that karyopherin alpha-3 may be involved in the nuclear transport system. KPNA3 ... Most nuclear proteins contain short basic amino acid sequences known as nuclear localization signals (NLSs). KPNA3, ... The transport of molecules between the nucleus and the cytoplasm in eukaryotic cells is mediated by the nuclear pore complex ( ...

*SLC7A9

... a disease that leads to cystine stones in the urinary system due to impaired transport of cystine and dibasic amino acids. ... This protein plays a role in the high-affinity and sodium-independent transport of cystine and neutral and dibasic amino acids ... Solute carrier family 7 (cationic amino acid transporter, y+ system), member 9 also known as SLC7A9 is a protein which in ... Heterodimeric amino acid transporter Solute carrier family "SLC7A9". Feliubadaló L, Font M, Purroy J, Rousaud F, Estivill X, ...

*Susan K. Gilmour

She has begun evaluating new approaches to disrupting the transport system through which polyamines can be imported into cancer ... Amino Acids. 2014 Mar;46(3):543-52. Epub 2013 Jul 25. http://www.limr.org/doc/Page.asp?PageID=DOC004139 http://www. ... with an inhibitor of the polyamine transport system not only blocks tumor growth but also promotes anticancer immune responses ...

*SLC7A3

... is a member of the system y+ family of transporters characterized by sodium-independent transport of cationic amino acids.[ ... "Entrez Gene: SLC7A3 solute carrier family 7 (cationic amino acid transporter, y+ system), member 3". Hosokawa H, Sawamura T, ... Cationic amino acid transporter 3 is a protein that in humans is encoded by the SLC7A3 gene. SLC7A3 ... Ito K, Groudine M (1997). "A new member of the cationic amino acid transporter family is preferentially expressed in adult ...
Looking for online definition of cystinuria type I in the Medical Dictionary? cystinuria type I explanation free. What is cystinuria type I? Meaning of cystinuria type I medical term. What does cystinuria type I mean?
Cystinuria is a disease of disrupted amino acid transport in the collecting ducts of the kidney failing to reclaim certain amino acids (cystine and the dibasic amino acids ornithine, lysine and arginine referred to as COLA). The increased urinary COLA concentrations reach saturation levels for cystine, which precipitates to form crystals and stones resulting in renal to urethral obstructions. Mutations in the SLC3A1 and SLC7A9 genes give rise to cystinuria in the vast majority of cystinuric humans, where the disease shows autosomal recessive or dominant inheritance (reviewed in Palacin et al. 2001; Chillaron et al. 2010).. Cystine calculi have been reported from at least 70 dog breeds, with increased incidence in several breeds (Ling et al. 1998; Osborne et al. 1999); in contrast, cystinuria is rarely seen in cats. We previously demonstrated autosomal recessively inherited cystinuria in ...
ABSTRACT: Human cystinotic fibroblasts were completely depleted of their accumulated intracellular free cystine within a 2-h time interval when exposed to culture medium containing between 1 and 5 mM mercaptoethylgluconamide. This cystine-depleting action of mercaptoethylgluconamide was observed with three different human cystinotic fibroblast cell lines and with all three cell lines, 2 mM mercaptoethylgluconamide was as effective as 1 mM cysteamine in depleting cells of their intracellular free cystine. Cell viability was excellent for cystinotic fibroblasts exposed to 2 mM mercaptoethylgluconamide for up to 6 days in duration. Mercaptoethylgluconamide (2 mM) was sufficiently stable under cell culture conditions such that a single addition of mercaptoethylgluconamide maintained cystine depletion in human cystinotic fibroblasts for at least a 4-day period. In contrast to cysteamine, 2 mM mercaptoethylgluconamide was not capable of depleting the cystine content of isolated cystinotic lysosomes, implying
The aim of this thesis is to better understand the regulation of the cystine/glutamate antiporter (system xc-) and its role in regulating neuronal survival and death. Expressed primarily on astrocytes, system xc- takes up cystine and releases glutamate in a 1:1 ratio. Cystine uptake is the rate-limiting step in glutathione synthesis, the brains main antioxidant. Glutamate released into the extrasynaptic space can regulate neuronal function; however excessive glutamate release can cause excitotoxicity. The dual actions of system xc- make it of interest in many neurodegenerative diseases where oxidative stress and excitotoxicity are involved. We investigated the regulation of system xc- in SOD1-G93A transgenic mouse model of ALS. We observed an increase in cystine uptake and glutamate release through system xc- in spinal cord slices of SOD1-G93A transgenic mice. We did not observe a change in the function of the main ...
Cystinuria is a condition characterized by the buildup of the amino acid cystine, a building block of most proteins, in the kidneys and bladder. As the kidneys filter blood to create urine, cystine is normally absorbed back into the bloodstream. People with cystinuria cannot properly reabsorb cystine into their bloodstream, so the amino acid accumulates in their urine.. As urine becomes more concentrated in the kidneys, the excess cystine forms crystals. Larger crystals become stones that may lodge in the kidneys or in the bladder. Sometimes cystine crystals combine with calcium molecules in the kidneys to form large stones. These crystals and stones can create blockages in the urinary tract and reduce the ability of the kidneys to eliminate waste through urine. The stones also provide sites where bacteria may cause infections.. Cystinuria affects approximately 1 in 10,000 people.. ...
It is critical to prevent stone formation in people with cystinuria as these stones can get very big very quickly. Not only do people with cystine stones undergo a high number of procedures and surgeries, but also compared to the more common stone types, their kidney function is more often impaired.. The biggest key to preventing cystine stones (as most others) is to maintain a very high fluid intake. In the case of cystine stones, we can estimate exactly how much a person needs to drink by their total daily cystine excretion, which can be obtained from a 24-hr urine collection - or better yet several. Once we know how much cystine, on average, a person makes, we can determine how much fluid intake is necessary.Typically, at least 3-4 liters of urine are required daily, and often much more than that. Variety helps to maintain such high intakes.. We also know that making the urine more alkaline (less acidic) is a cornerstone of therapy in patients with cystinuria. Some patients with cystine ...
The first cystine stone was identified in 1810 by Wollaston, who called it cystic oxide.65 Cystinuria is an autosomal recessive disease characterised by renal and intestinal dibasic amino acid transport defects affecting cystine, ornithine, arginine, and lysine, of which cystine is the least soluble and therefore the most likely to precipitate as a stone.66 Cystinuria type I has now been shown to be caused by a mutation in the gene SLC3AI, which encodes the rBAT protein.67 The M467T mutation appears to be particularly important, but there are several other minor variants that may also be relevant.68 Regardless of cause, the primary goal of medical management is to prevent the formation of new stones by reducing the cystine concentration to below its upper limit of solubility. The precise solubility limits are unclear, with a variety of possibilities cited: urine solubility of 250 mg/litre (200 μmol/litre) at pH 5.0, rising to 500 mg/litre (410 μmol/litre) at ...
The transport of cystine has been investigated in mouse peritoneal macrophages cultured in vitro. The transport activity for cystine was very low in freshly isolated macrophages but was potently induced during culture in the presence of bacterial lipopolysaccharide (LPS) at concentrations as low as 0.1 ng/ml. The transport activity for cystine was enhanced when the cells were incubated with tumour necrosis factor-alpha (TNF-alpha), but not with interferon-gamma (IFN-gamma) or interleukin-1. IFN-gamma was rather repressive in the induction of the activity by LPS or TNF-alpha. The transport activity for cystine induced by LPS has been characterized. Cystine was transported mainly by Na(+)-independent system and the uptake of cystine was inhibited by extracellular glutamate and homocysteate, but not by aspartate, indicating that the transport of cystine in macrophages treated with LPS is ...
Cystinuria is a rare genetic disorder characterized by recurrent, painful kidney stones, primarily composed of cystine, the dimer of the amino acid cysteine (Sumorok and Goldfarb, 2013). Using a mouse model of cystinuria, we have recently shown that administration of drugs that increase cystine solubility in the urine can be a novel therapeutic strategy for the clinical management of the disease (Zee et al., 2017). There is a large unmet need in the field for developing new drugs for cystinuria. To that end, here we describe a simple in vitro cystine solubility assay that is amenable for screening compounds to identify potential drugs that may influence cystine solubility. The assay includes preparing a supersaturated solution of cystine, incubating this solution with drug(s) of choice, and finally using high pressure liquid chromatography-tandem mass spectrometry (HPLC-MS/MS) to quantify the amount of cystine precipitated under various conditions.
Several studies have shown that the cRNA of human, rabbit, or rat rBAT induces in Xenopus oocytes sodium-independent, high affinity uptake of L-cystine via a system b0,(+)-like amino acid exchanger. We have shown that mutations in rBAT cause type I cystinuria (Calonge, M. J., Gasparini, P., Chillarón, J., Chillón, M., Gallucci, M., Rousaud, F., Zelante, L., Testar, X., Dallapiccola, B., Di Silverio, F., Barceló, P., Estivill, X., Zorzano, A., Nunes, V., and Palacín, M. (1994) Nat. Genet. 6, 420-425; Calonge, M. J., Volipini, V., Bisceglia, L., Rousaud, F., De Sanctis, L., Beccia, E., Zelante, L., Testar, X., Zorzano, A., Estivill, X., Gasparini, P., Nunes, V., and Palacín, M. (1995) Proc. Natl. Acad. Sci. U. S. A. 92, 9667-9671). Apart from oocytes, no other expression system has been used for transfection of functional rBAT activity. Furthermore, the b0,(+)-like transport activity has not been clearly described in the ...
Cystinuria definition, an inherited metabolic disorder that results in the excessive excretion of certain amino acids, especially cystine, in the urine. See more.
Background: Cystinuria is the most common inherited cause of recurrent kidney stones (1). Its principal disorder is impaired reabsorption of cystine in the proximal renal tubule. As a result, large amounts of cystine are excreted in urine, kidney stones form because cystine is poorly soluble, and the patient experiences many urologic interventions. Measures to prevent stone formation include changing the diet to reduce cystine excretion, diluting urine to a lower cystine concentration, and making the urine alkaline to improve cystine solubility. Cystine-binding thiol drugs also improve cystine solubility but need to be discontinued because of adverse effects in 30% to 60% of cases (1) ...
Find SLC3: Heavy Subunits of the Heteromeric Amino Acid Transporter Family research area related information and SLC3: Heavy Subunits of the Heteromeric Amino Acid Transporter Family research products from R&D Systems. Learn more.
1. By using ion-exchange columns coupled to a sensitive automated Sakaguchi reaction, in addition to the normal ninhydrin reaction for amino acids, homoarginine, a guanidino homologue of arginine, was found in the plasma and urine of both normal and cystinuric individuals.. 2. In all seven cystinuric subjects studied, urinary excretion of homoarginine was approximately ten times that found in normal subjects; the plasma levels of this amino acid were considerably reduced relative to normal individuals.. 3. Homoarginine in the plasma can be derived either metabolically from lysine or from dietary sources.. 4. In normal subjects homoarginine was cleared at a higher rate than arginine. On the other hand, the clearance of arginine exceeded that for homoarginine in the majority of cystinuric subjects although the values obtained for homoarginine indicate that the defect in amino acid transport ...
BioAssay record AID 552756 submitted by ChEMBL: Antinephropathic cystinosis activity in human cystinotic fibroblast assessed as decrease in cystine level at 50 uM after 24 hrs by Bradford reagent method.
Cystinuria is a health condition characterized by increased concentration of cystine and other dibasic amino acids in the urine. It most commonly occurs in young adults between the age of twenty and thirty years, although some individuals may present as children or in adolescence.
Learn about the inherited disease cystinuria, an excess of cystine/cystine kidney stones in the urine. Learn how you get kidney stones through inheritance, passing kidney stones, what causes kidney stones, symptoms of kidney stones, kidney stone pain, and kidney stone treatment.
Looking for Cystine storage disease? Find out information about Cystine storage disease. A congenital metabolic disorder involving sulfur-containing amino acids, usually cystine; characterized by deposits of cystine crystals in the body organs Explanation of Cystine storage disease
In this fourth and final blog in a series about preventable inherited diseases in the Labrador retriever (see previous blogs here; part one, part two, and part three), we will examine a potentially life-threating urinary disease and a cause of blindness in dozens of dog breeds including the Labrador. Cystinuria...
The steady-state level of α1(I) collagen mRNA is regulated by amino acid availability in human lung fibroblasts. Depletion of amino acids decreases α1(I) collagen mRNA levels and repletion of amino acids induces rapid re-expression of α1(I) mRNA. In these studies, we examined the requirements for individual amino acids on the regulation of α1(I) collagen mRNA. We found that re-expression of α1(I) collagen mRNA was critically dependent on cystine but not on other amino acids. However, the addition of cystine alone did not result in re-expression of α1(I) collagen mRNA. Following amino acid depletion, the addition of cystine with selective amino acids increased α1(I) collagen mRNA levels. The combination of glutamine and cystine increased α1(I) collagen mRNA levels 6.3-fold. ...
Looking for online definition of cystine lyase in the Medical Dictionary? cystine lyase explanation free. What is cystine lyase? Meaning of cystine lyase medical term. What does cystine lyase mean?
This mild shampoo is formulated to stimulate protective base two essential assets to the health of the hair: a precursor of cystine essential constituent of keratin and vitamin B6 to optimize the use of Cystine. This association helps stimulate hair growth and improve quality while controlling sebum secretion.. Cystine B6 Shampoo. Provitamin B5 associated with anti-pollution complex reinforces the strength of the hair and protects them from external pollutants.. With its gentle cleansing base and its pleasant scent Cystine B6 shampoo cleans hair without assaulting them and facilitates their styling.. Seboregulator, restructuring, antioxidant, detoxifier, and anti-scale pollution. Prepares the scalp treatments fall. Gentle washing base and fragrant, easy styling, gives strength, vigor, vitality and shine to hair. Hypoallergenic.. Shelf Life after opening: 12 months.. • Indications of shampoo Cystine B6. hair loss, additional treatments fall. Protection from external aggression. Frequent ...
Renal tubular reabsorption of cystine and lysine were studied in the isolated perfused rat kidney to bridge the gap between in vivo clearance studies, and in vitro transport studies of tubule fragments, cells, and brush-border membranes. Lysine was reabsorped by a saturable transport system shared by the dibasics. Cystine was also reabsorbed by a saturable transport system, which was shared in part by the dibasics (maximum inhibition 30%). The lysine threshold (Fmin) was 0.9 mumol.min-1.g-1, with a tubular maximum (TM) of 2.4 mumol.min-1.g-1. The cystine Fmin was 0.06 mumol.min-1.g-1; the TM could not be estimated because it was above the limit of cystine solubility. There was no evidence of cystine ,secretion.- The gamma-glutamyltransferase inhibitor, AT-125, decreased cystine excretion, but only in the presence of glutathione, glycine, glutamate, and the diabasic amino acids. This suggests ...
KEGG Orthology (KO) [BR:nme00001] Environmental Information Processing Membrane transport 02010 ABC transporters [PATH:nme02010] NMB0789 amino acid ABC transporter ATP-binding protein K10010 ABC.CYST.A; cystine transport system ATP-binding protein [EC:3.6.3.-] Enzymes [BR:nme01000] 3. Hydrolases 3.6 Acting on acid anhydrides 3.6.3 Acting on acid anhydrides to catalyse transmembrane movement of substances 3.6.3.- NMB0789 amino acid ABC transporter ATP-binding protein K10010 ABC.CYST.A; cystine transport system ATP-binding protein [EC:3.6.3.-] Transporters [BR:nme02000] ABC Transporters, Prokaryotic Type Phosphate and amino acid transporters Cystine transporter [MD:M00234] ...
System xc− represents an intriguing target in attempts to understand the pathological states of the central nervous system. Also called a cystine-glutamate antiporter, system xc− typically functions by exchanging one molecule of extracellular cystine for one molecule of intracellular glutamate. Nonvesicular glutamate released during cystine-glutamate exchange activates extrasynaptic glutamate receptors in a manner that shapes synaptic activity and plasticity. These findings contribute to the intriguing possibility that extracellular glutamate is regulated by a complex network of release and reuptake mechanisms, many of which are unique to glutamate and rarely depicted in models of excitatory signaling. Because system xc− is often expressed on non-neuronal cells, the study of cystine-glutamate exchange may advance the emerging viewpoint that glia are active contributors to information processing in the brain. It is noteworthy that ...
0028] Based on the results, it can reasonably be projected that soluble N.sup.α-long chain alkanoyl dibasic amino acid alkyl ester salts will provide synergistic anti-microbial activity when combined with a glycerol mono-fatty acid ester. It would seem that in order to obtain the practical benefits of the synergism between an N.sup.α-long chain alkanoyl dibasic amino acid alkyl ester salt and a glycerol mono-fatty acid ester, the solubility of the N.sup.α-long chain alkanoyl dibasic amino acid alkyl ester salt would need to be sufficient to release a concentration of N.sup.α-long chain alkanoyl dibasic amino acid alkyl ester cation which is greater than microbial inhibitory concentration of that ion in water at RT. Based on the data, it would seem that the absolute minimum solubility required would be approximately 5 ppm in water at RT. ...
The shared transport system for uptake of L-cystine and L-lysine was examined in isolated rat renal brush-border membrane vesicles for the ionic requirements for activation of the system. No requirement for sodium was seen for either cystine or lysine influx. However, the efflux of lysine from the vesicle was stimulated by Na+. Therefore, the transport system appears to be asymmetric in its requirement for sodium. Two different divalent cations were used in the membrane isolations which resulted in different responses of cystine uptake to the electrogenic movement of K+ out of the vesicle. Membranes prepared by Mg-aggregation showed no stimulation of cystine influx by the imposition of a transient interior negative potential while vesicles prepared by Ca-aggregation did respond to electrogenic stimulation by an outwardly directed K-diffusion potential in the presence of valinomycin. Lysine influx was stimulated by ...
Scientists studying the rare and deadly genetic disease cystinosis, have shown that transplantation of adult bone marrow stem cells appears to virtually halt the disease in a mouse model. Cystine is a byproduct of the lysosomal breakdown of cellular components the body no longer needs. Normally, cystine is shunted out of cells, but in cystinosis a gene defect of the lysosomal cystine transporter causes cystine to build up, forming crystals that are especially damaging to the kidneys and eyes. The only available drug to treat cystinosis, cysteamine, while slowing the progression of kidney degradation, does not prevent it, and end-stage kidney failure is inevitable. "Cysteamine must be given every six hours, so children have to be woken up each night to take this drug, which has unpleasant side effects, and many others to treat various symptoms," said Dr. Stephanie Cherqui, the senior author of the study. "So although there is treatment, it is difficult treatment that does not cure the ...
L-Cystine is a covalently linked dimeric nonessential amino acid formed by the oxidation of cysteine. Two molecules of cysteine are joined together by a disulfide bridge to form cystine. Cystine is a chemical substance which naturally occurs as a deposit in the urine, and can form a calculus (hard mineral formation) when deposited in the kidney. The compound produced when two cysteine molecules linked by a disulfide (S-S) bond. Cystine is required for proper vitamin B6 utilization and is also helpful in the healing of burns and wounds, breaking down mucus deposits in illnesses such as bronchitis as well as cystic fibrosis. Cysteine also assists in the supply of insulin to the pancreas, which is needed for the assimilation of sugars and starches. It increases the level of glutathione in the lungs, liver, kidneys and bone marrow, and this may have an anti-aging effect on the body by reducing age-spots etc.. ...
Red or orange stones probably contain uric acid. Likewise, stones that do not show up on simple abdominal flat plate x rays are probably uric acid. These are easy to prevent, and recurrences are unnecessary.. Allow me to elaborate on this. Pure uric acid stones are almost all due to an excessively acid urine pH, and will stop if you raise that pH. Potassium citrate tablets, 10 mEq size, 2 twice daily is almost always enough. Sometimes it takes 2 tabs 3 times a day. Crystal light lemonade has in one liter about the same amount of alkali as two of the tablets. I could say that there is little excuse for another uric acid stone.. If stones contain uric acid and other crystals, those other crystals need to be dealt with on their own. They may not respond to higher urine pH, and could be worsened.. Yellow stones are probably cystine, and due to cystinuria, a complex disease with its own special treatments. ...
cystine definition: a nonessential amino acid, CH(NH)S(COOH)Origin of cystine cyst(o)- + -ine: so named from having been found first in urinary calculi...
Initial treatment is with adequate hydration, alkalization of the urine with citrate supplementation or acetazolamide, and dietary modification to reduce salt and protein intake (especially methionine). If this fails then patients are usually started on chelation therapy with an agent such as penicillamine.[10][11] Tiopronin is another agent. Once renal stones have formed, however, the first-line treatment is ESWL (Extracorporeal shock wave lithotripsy). If ESWL do not work efficiently surgery can be necessary. Both endoscopic surgery and conventional open-abdominal surgery have proven to be effective treatment modalities for patients with more advanced disease. Adequate hydration is the foremost aim of treatment to prevent cysteine stones. The goal is to increase the urine volume because the concentration of cystine in the urine is reduced which prevents cystine from precipitating from the urine and forming stones. People with cystine stones should consume 5 to 7 liters a day. The rationale ...
In this report, an 8-year-old girl is presented with the complaint of progressive night blindness. The authors have performed eye funduscopy, which showed chorioretinal atrophy in gyrate shape. A high level of plasma ornithine was determined. Urinary excretion of ornithine as well as lysine and cystine were increased. Patient was treated with high dose pyridoxine supplement (500 mg/dl). The night blindness condition of the patient improved. After 1 month of pyridoxine therapy ornithine level of her plasma was successfully reduced and blindness improved.
The use of hematopoietic stem cell transplantation (HSCT) to treat leukemic disorders is well established in the treatment of systemic diseases. Similarly, the use of HSCT to treat genetic diseases, caused by the lack or abnormal expression of a gene, such as in sickle cell anemia, is another clinical indication for this form of cell-based therapy. In fact, the use of HSCT to treat this inherited disease is associated with a better rate of engraftment, survival, and decreased complications. Therefore, the use of HSCT to treat genetic inherited diseases has a great potential. The work reported by Cherqui et al.1 demonstrated that the use of HSCT to treat the ophthalmic manifestations is promising and, moreover, shows that the eye can be used to monitor success of engraftment in monitoring clinical response and to dissect mechanisms of action. Cystinosis is caused by a deficiency in the lysosomal cystine transporter, cystinosin (CTNS gene), resulting in cystine crystal ...
Cystinosis is a lysosomal storage disease characterized by the abnormal accumulation of the amino acid cystine. It is a genetic disorder that typically follows an autosomal recessive inheritance pattern. It is a rare autosomal recessive disorder resulting from accumulation of free cystine in lysosomes, eventually leading to intracellular crystal formation throughout the body. Cystinosis is the most common cause of Fanconi syndrome in the pediatric age group. Fanconi syndrome occurs when the function of cells in renal tubules is impaired, leading to abnormal amounts of carbohydrates and amino acids in the urine, excessive urination, and low blood levels of potassium and phosphates. Cystinosis was the first documented genetic disease belonging to the group of lysosomal storage disease disorders. Cystinosis is caused by mutations in the CTNS gene that codes for cystinosin, the lysosomal membrane-specific transporter for ...
On March 12, 2013, our biochemical laboratory started to use an optimized protocol for cystine determination in white blood cells for test code 4627. This protocol change minimizes the loss of cystine during sample preparation. The normal cystine range is not affected by this change; however, as a result of the protocol change, the cystine level reported may be increased in cystinosis patients. This apparently elevated level may lead to concern for patient non-compliance when compared with previous results. Please take this method change into consideration when monitoring the patients cystine levels ...
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System xc- is a heterodimeric plasma membrane transporter involved in the exchange of intracellular glutamate for extracellular cystine. As such, this transporter plays a critical role in the production of the antioxidant glutathione. Previous studies in our lab have demonstrated that there is an increase in cell surface expression within ten minutes of exposure to H2O2 in confluent U138MG human glioma cells. The study described herein sought to begin to characterize the mechanism by which H2O2 regulates the trafficking of xCT. We hypothesized that Akt signaling is necessary for H2O2-mediated trafficking of of xCT. A significant increase in Akt phosphorylation was observed in U138MG cells following ten-minute exposure to 3 mM H2O2 compared to vehicle-treated cells using western blot analysis. Treatment with the Akt inhibitor 10- DEBC (2.5µM) for 30 minutes prior to and during H2O2 exposure resulted in a decrease in H2O2-induced phosphorylation of Akt at ...
OBJECTIVES: To perform an in vitro study analyzing the possibilities in therapeutic strategies for an effective treatment of cystine stones. METHODS: Artificial stones made of cystine [Bon(n)-Stones] with a ball-shaped size of 0.9 cm in diameter were
BACKGROUND: Cystinosis is an autosomal recessive disorder characterized by intralysosomal cystine accumulation. Growth retardation is more pronounced in cystinosis than in other chronic kidney diseases and is mostly not corrected by cysteamine. METHO
Cystinosis has been classified as a lysosomal storage disorder on the basis of cytologic and other evidence pointing to the intralysosomal localization of stored cystine. Cystinosis differs from the other lysosomal diseases inasmuch as acid hydrolysis, the principal enzyme function of lysosomes, is not known to play a role in the metabolic disposition of cystine. The fact that plasma levels are well below saturation indicates that the defect is a cellular one. Within the cell, cystine is compartmentalized with acid phosphatase and is membrane-bound as demonstrated by electron microscopy. Ferritin accumulates in the same organelle which appears to be the lysosome. (219800) ...
Some patients may experience drug fever, a marked febrile response to penicillamine, usually in the second to third week following initiation of therapy. Drug fever may sometimes be accompanied by a macular cutaneous eruption. In the case of drug fever in patients with Wilsons disease or cystinuria, penicillamine should be temporarily discontinued until the reaction subsides. Then penicillamine should be reinstituted with a small dose that is gradually increased until the desired dosage is attained. Systemic steroid therapy may be necessary, and is usually helpful, in such patients in whom drug fever and rash develop several times.. In the case of drug fever in rheumatoid arthritis patients, because other treatments are available, penicillamine should be discontinued and another therapeutic alternative tried since experience indicates that the febrile reaction will recur in a very high percentage of patients upon readministration of penicillamine.. The skin and mucous membranes should be ...
...Researchers in France have hit on a novel method to help kidney stone ...Kidney stones represent a major medical problem in the western and dev...Worldwide approximately 1:7000 births are affected by cystinuria the ...Cystine stones of which there are two forms are composed of tiny mic...,More,effective,kidney,stone,treatment,,from,the,macroscopic,to,the,nanoscale,biological,biology news articles,biology news today,latest biology news,current biology news,biology newsletters
Amy E. Krambeck, John C. Lieske Mayo Clinic December 2, 2014 Comment We read with interest the recent Clinical Practice guidelines from the American College of Physicians (ACP) for the Prevention of Kidney Stones in Adults. The recommendations for drinking at least 2 L of fluid per day and the use of thiazide diuretics, citrate, or allopurinol when fluids alone are insufficient mirror recent guidelines released by the American urological Association (AUA). However, several features of the ACP recommendations are in disagreement with those of the AUA. For example, baseline stone composition is not recommended by ACP, nor is 24-hour urine analysis for stone risk factors. Kidney stone analysis by infrared spectroscopy is relatively inexpensive and very precise and, in our opinion, essential to properly diagnose the form of kidney stone disease. For example, a thiazide diuretic would not be helpful for patient with uric acid kidney stones or someone with cystinuria, both of which can be ...
Penicillamine is a chelating agent used in the treatment of Wilsons disease. It is also used to reduce cystine excretion in cystinuria and to treat patients with severe, active rheumatoid arthritis unresponsive to conventional therapy. Penicillamine is used as a form of immunosuppression to treat rheumatoid arthritis. It works by reducing numbers of T-lymphocytes, inhibiting macrophage function, decreasing IL-1, decreasing rheumatoid factor, and preventing collagen from cross-linking. Its use in Wilsons disease, a rare genetic disorder of copper metabolism, relies on its binding to accumulated copper and elimination through urine ...
Penicillamine is a chelating agent used in the treatment of Wilsons disease. It is also used to reduce cystine excretion in cystinuria and to treat patients with severe, active rheumatoid arthritis unresponsive to conventional therapy. Penicillamine is used as a form of immunosuppression to treat rheumatoid arthritis. It works by reducing numbers of T-lymphocytes, inhibiting macrophage function, decreasing IL-1, decreasing rheumatoid factor, and preventing collagen from cross-linking. Its use in Wilsons disease, a rare genetic disorder of copper metabolism, relies on its binding to accumulated copper and elimination through urine ...
Biologic samples will be stored in the biobank from well characterized patients with primary hyperoxaluria, cystinuria, APRT deficiency, and Dent disease, and from their family members, for use in future research. This will help to advance our understanding of disease expression and the factors associated with kidney injury in these four diseases with the overall goal of developing new treatments to preserve kidney function and reduce nephrocalcinosis and stone formation ...
ACC 2011 - NEW ORLEANS -- High levels of the amino acid cystine in the blood may indicate an increase in oxidative stress that can cause atrial fibrillation in patients following cardiac catheterization procedures, researchers suggested here.. Keywords: ACC Annual Scientific Session ...
Thiola is used to prevent kidney stones, which may develop due to too much cystine in the urine (cystinuria). This medicine works by removing the extra cystine from the body....
Adenine phosphoribosyltransferase (APRT) deficiency, cystinuria, Dent disease, familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC), and primary hyperoxaluria (PH) are rare but imp
Retrophin, Inc. is a biopharmaceutical company focused on the development, acquisition and commercialization of therapies for the treatment of serious, catastrophic or rare diseases. It sells three products, including Chenodal (chenodeoxycholic acid), Cholbam (cholic acid) and Thiola (tiopronin). Its Chenodal is approved in the United States for the treatment of patients suffering from gallstones in whom surgery poses an unacceptable health risk due to disease or advanced age. Chenodal has also been care for cerebrotendinous xanthomatosis (CTX) patients. Its Cholbam is approved in the United States for the treatment of bile acid synthesis disorders due to single enzyme defects and is further indicated for adjunctive treatment of patients with peroxisomal disorders. Its Thiola is approved in the United States for the prevention of cystine (kidney) stone formation in patients with severe homozygous cystinuria. Its product candidates include Sparsentan, RE-024, ...
Peak times for clinical expression of cystinuria are usually during the second and third decades of life (the disease may appear as early as in the first year or as late as in the ninth decade of life). Renal cholics with hematuria as a consequence of nephrolithiasis, urinary tract infections, and obstructive uropathies are common manifestations. Hypertension and renal failure occur occasionally. Onset of neurologic symptoms, such as progressive loss of motor and cognitive milestones, seizures, spasticity, and ataxia, have been reported to occur in early childhood. Further deterioration of neurologic status often coincides with urinary tract infections. Alteration of intestinal flora by ...
Philip Anderson Shaffer was born in Martinsburg, West Virginia. He graduated from the University of West Virginia with a B.A. in 1900 and from Harvard University with a Ph.D. degree in 1904. At Harvard, Shaffer worked with Otto Folin and became interested in methods for the determination of organic substances in blood, urine, and milk. For a time, Shaffer served as a research biological chemist at the McLean Hospital in Waverly, Massachusetts, and from 1904 to 1910 he was an instructor in pathological chemistry at Cornell University Medical College. He then became professor of biological chemistry at the Washington University School of Medicine, St. Louis, Missouri, where he built up a strong department of physiological chemistry. Shaffers scientific work was varied in character. He studied metabolism in disease, nutrition in typhoid fever, protein metabolism in exophthalmic goiter, and protein metabolism in cystinuria. His studies on antiketogenesis were especially noteworthy and appeared in a ...
Acetazolamide is a carbonic anhydrase inhibitor used to treat symptoms of altitude sickness. It also belongs to the class of diuretics and it is effective in the treatment of glaucoma, epileptic attacks, intracranial hypertension, cystinuria, dural ectasia. | Ph24h.com
Giacopo, Andrea Di; Rubio-Aliaga, Isabel; Cantone, Alessandra; Artunc, Ferruh; Rexhepaj, Rexhep; Frey-Wagner, Isabelle; Font-Llitjós, Mariona; Gehring, Nicole; Stange, Gerti; Jaenecke, Isabel; Mohebbi, Nilufar; Closs, Ellen I; Palacín, Manuel; Nunes, Virginia; Daniel, Hannelore; Lang, Florian; Capasso, Giovambattista; Wagner, Carsten A (2013). Differential cystine and dibasic amino acid handling after loss of function of the amino acid transporter b0,+AT (Slc7a9) in mice. American Journal of Physiology. Renal, Fluid and Electrolyte Physiology, 305(12):F1645-F1655.. Mariotta, Luca; Ramadan, Tamara; Singer, Dustin; Guetg, Adriano; Herzog, Brigitte; Stoeger, Claudia; Palacín, Manuel; Lahoutte, Tony; Camargo, Simone M R; Verrey, François (2012). T-type amino acid transporter TAT1 (Slc16a10) is essential for extracellular aromatic amino acid ...
Levodopa (L-DOPA) is the naturally occurring precursor amino acid for dopamine and the main therapeutic agent for neurologic disorders due to dopamine depletion, such as Parkinsons disease. Levodopa absorption in small intestine has been suggested to be mediated by the large neutral amino acids transport machinery, but the identity of the involved transporters is unknown. Clinically, co-administration of levodopa and dietary amino acids is avoided to decrease competition for transport in intestine and at the blood brain barrier. Levodopa is routinely co-administered with levodopa metabolism inhibitors (dopa decarboxylase and cathechol-o-methyl transferase inhibitors) that share structural similarity with levodopa. In this systematic study involving Xenopus laevis oocytes and MDCK epithelia expression systems and ex vivo ...
The recent humanitarian release of Libyan citizen Abdalbaset al-Megrahi from prison in Greenock, Scotland, because of his poor health, and his subsequent "heros welcome" in Libya is strikingly incongruous when compared with the tragic fate of Fathi al-Jahmi, a Libyan prisoner who also suffered from poor health, including coronary artery disease, congestive heart failure, hypertension, and diabetes.. Libyan authorities held Mr. al-Jahmi prisoner in Tripoli on two occasions for a total of six and a half years. His "crime"? The peaceful exercise of his fundamental rights of freedom of expression and association. He advocated for democratic reforms and free elections, as have many other Libyan citizens whose outspoken opinions have led to their imprisonment. Additionally, Mr. al-Jahmi had the audacity to directly criticize the Libyan government and its leader of 40 years, Colonel Muammar al-Quaddafi.. Mr. al-Jahmis second arrest took place in March 2004. In February 2005, a medical doctor ...
Changes in urinary and plasma taurine and amino acids have been evaluated in trained runners competing in the Rotterdam Marathon, 1998, both immediately after completing the event and 24h after recovery. There were significant changes in the urinary amino acids excretion, the majority showing a significant decrease both immediately at the completion of the Marathon and after 24h recovery. In contrast urinary taurine excretion increased immediately post Marathon, although not significantly as the range of results was wide. Such changes in urinary taurine correlated with percentage changes in plasma creatine kinase both immediately post race, (r = 0.972, P , 0.001), and 24h later (r = 0.872, P , 0.001), possibly indicating that the source of the taurine was muscle. Significant correlations between the individual values for urinary and plasma amino acids in all of the athletes were calculated for taurine (r = ...
Author Summary Insulin-dependent type 1 diabetes (T1D) is characterized by elevated blood sugar, lymphocytic infiltration into the islets of Langerhans and T cell destruction of beta (β) cells. β cells produce insulin whose function is to maintain and regulate glucose hemostasis. However, in vivo, the numbers of antigen specific T cells that migrate to the islets to cause T1D, the engagement of such T cells with β cells at the immunologic synapse and the molecules expressed at the synapse are not clear. Using a transgenic model of virus induced T1D, a panel of viruses with CD8 T cell epitope mutations and in situ tetramer hybridization, we note of the total CD8 T cells infiltrating the islets, only 1-2% are antigen specific recognizing the immunodominant virus CD8 T cell epitope expressed on β cells. Immunohistochemical analysis of the synapse found between antigen specific CD8 T cells and β cells displays attachment by LFA-1 and presence of perforin, the molecule indicative of lytic activity.
So what can we learn from all this? Does this study - or rodent research in general - have much relevance for those of us who lack tails, fuzzy ears, and adorable pink noses?. The answer is an equivocal "yes and no." One reason mice are a favored lab animal is that they share so many genes with humans - 15,187 of them, to be exact. Heck, it was only 90 million years ago that we split from a common ancestor. Ive met Okinawans older than that!. But that doesnt mean gene expression always works the same, or that the causes and progression of disease are identical across species. Even when high-fat diets catapult mice towards diabetes, for instance, their markers for disease dont always resemble ours. Unlike metabolically damaged humans, who tend to have rock-bottom HDL cholesterol and rising triglycerides, some mice experience higher HDL and unchanged (or reduced!) triglycerides when eating the diets that make them diseased (PDF). This points to some clear differences between how humans and mice ...
A warning from our founders: Nutritionists used to think these 2 vegetables were healthy!!!. But a new discovery revealed 2 vegetables you should almost NEVER eat, because they spike your blood sugar, cause Type 2 Diabetes and make your body store annoying belly fat!. Thats right - 2 common vegetables that most people eat 2-3 times per week make your belly bulge over your belt, and complicate or even cause Type 2 Diabetes.. On the next page youll see a free presentation that explains what these 2 vegetables are, and why they add to ugly, stubborn body fat. Plus, youll find out how these discoveries lower your blood sugar, reverse Type 2 Diabetes, and make your belly fat vanish!. AVOID these 2 Vegetables to Lower Your Blood Sugar & Shrink Belly Fat. ...
Question - Pancreatic parenchymal abnormalities consisting of hyperechoic strands ,hyperechoic focci, lobularity noted in entire pancreas consistent with mild chronic pancreatitis. Suggestions?. Ask a Doctor about when and why Ultra sound is advised, Ask a Radiologist
Small non-coding RNAs, are quickly becoming cornerstones in disease diagnosis and therapy, including microRNAs (miRNA) being discovered as potent biomarkers for a number of tumors. A paper published in Molecules this month looks into the possibility of miRNAs becoming a sensitive, reliable and non-i
Researchers at UCLA are reporting on an amazing achievement of having a completely paralyzed man voluntarily walk using an exoskeleton coupled with a non-i
The protein composition of Collagen Type I & III consist of nineteen amino acids responsible for growth and maintenance of the body with unusually high percentages of glycine, proline, hydroxyproline, all of which are found in the skin and muscle. This is what make Collagen M.D. Advanced Connective Tissue Formula effective in those areas. ...
Joining the posted submitter club, suasfan22 writes with a bit in Wired about the use of a drone by Libyan Rebels. From the article: The Libyan revolutionaries are more of a band of enthusiastic amateurs than experienced soldiers. But it turns out the rebels have the kind of weaponry usually posses...
This study demonstrates how an identity was constructed on the Libyan Youth Movements Facebook page by the pages authors. The study uses a data set collected during the opening weeks of the Libyan revolution, using data almost entirely from the month of February, 2011. The authors assume that new media allows for the increased expression of identity and that discourse analysis can be used to uncover the identity/identities of the LYM. Following in the footsteps of the Constructivist authors we endeavor to uncover the identity of the LYM which will allow for the prediction of the LYMs goals.. ...
A research group has identified specific mutations in a gene on chromosome 15 called KBTBD13 that cause a type of nemaline myopathy (NM), a disease in which thread- or rod-like ("nemaline") material forms clumps in affected muscle. The newly identified mutations cause type 6 NM. A number of mutations in genes associated with other subtypes of the disease already have been identified. ...
The thought that comes to mind is that what makes one person have a leaky gut for hours and another person has a leaky gut for just minutes. This leads to our understanding that a person must have a genetic predisposition to a disease. We cant fix predisposition because that it what we get from our parents. But it is only with the environmental trigger, however, that a person actually becomes sick. Fasano and others believe that many autoimmune diseases are affected by food sensitivities and changes in the microbiome at an early age. The leaky gut has been associated with other autoimmune diseases such as type I diabetes.[i] Type I diabetes is one such autoimmune disease which a patients own immune system attacks the insulin producing beta cells. Without insulin, our bodies are unable to process sugars and convert them to a storage form, fat. The link between how the leaky gut triggers immune reactions that attack the pancreas in some people and cause type I diabetes and in others creates ...
There has been studies and research looking into Type 2 diabetes and finding that it may be the result of the bodys genetics as a way to survive winters without food and "stocking up" during summer months, etc. There was research into Native American Indians that showed evidence that genetics has led to the body actually allowing those who are "Type 2" prone to actually have survived in the past due to this, etc. Well, anyhow, this is controversial at this point. But a recent look at statistics in the U.S. has shown that while obesity in the U.S. has grown DRAMATICALLY in the last 2 years, there is NO increase in Type 2 diabetes. What this indicates is that obesity doesnt CAUSE Type 2 diabetes in any way...but rather, that the genetics that lead to the development of Type 2 diabetes ALSO make people prone to obesity. In other words, obesity is NOT a cause of Type 2 diabetes...but rather a co-symptom of the genetics that lead to Type 2 diabetes. This is good news and bad. First, it means that ...
BetaFat is a 2-arm, unblinded study to compare gastric banding to treatment with metformin over a 24-month period in moderately obese adults with pre- or mild type 2 diabetes. The primary outcome will be change in β-cell compensation for insulin resistance, which the investigators will compare between groups. Secondary analyses will include other potential markers of β-cell health and potential mediators of treatment-specific effects. The main focus will be on mediators related to obesity. Clinically, the project will serve as a test of concept for use of gastric banding relatively early in the spectrum of obesity and β-cell disease. Biologically, the results will provide crucial information on potential mediators of β-cell failure and its arrest or reversal in the context of obesity. Those mediators will guide the development of more effective treatment and monitoring for the β-cell disease that causes type 2 diabetes ...
So, A review was done on Gary Taubes new book. Albeit from someone who hasnt actually read it. Its more a review of snippets of information Taubes has made available on his blog to market the book. There was a really good discussion regarding Taubes work on StrengthCoach which Chi was a part of and made some very interesting points, refuting a lot of things that the likes of Taubes or Eades mention a lot i.e. theres no evidence to suggest elevated blood sugar levels cause type 2 diabetes therefore the its just an assumption that carbs cause elevated sugar levels which cause diabetes..... Also claims protein can spike insulin just as much if not more than carbs and that a high insulin response isnt a bad thing.... This was on the SC forum though, best to keep it to the blog post. Its worth while to state that Chi IS an advocate of better food choices he just says that there is nothing to show that carbs are any worse or better than any other macro nutrient and most of the Taubes or Eades ...
Fulfilling the promise of Mendelian randomization Joseph Pickrell doi: http://dx.doi.org/10.1101/018150 Many important questions in medicine involve questions about causality, For example, do low levels of high-density lipoproteins (HDL) cause heart disease? Does high body mass index (BMI) cause type 2 diabetes? Or are these traits simply correlated in the population for other reasons? A popular…
Researchers at Mount Sinai School of Medicine have identified how a gene for a protein that can cause Type 2 diabetes, also possibly kills nerve cells in the brain, thereby contributing to Alzheimers disease.
China Nutritional Ingredient L-Cystine (CAS 56-89-3), Find details about China L-Cystine, Amino Acids from Nutritional Ingredient L-Cystine (CAS 56-89-3) - Hefei Reachever Import and Export Limited Company
Libya footed the bill for medical evidence that suggested the Lockerbie bomber had just 3 months to live, the Sunday Telegraph reports. The Libyan government pushed doctors to make the prognosis, required... World News Summaries. | Newser
iframe src="https://biblio.ugent.be/publication?q=parent+exact+%22LIBYAN+JOURNAL+OF+MEDICINE%22&embed=1&hide_pagination=1&hide_info=1&hide_options=1&hide_cluster=1" ...
The cash-starved Libyan rebel government received emergency money in the final weeks before their advance into Tripoli -- a $100 million cash donation from the government of Turkey.
A majority of Libyans (54%) approve of U.S. leadership and favor military aid from the West. More than six in 10 consider al-Qaeda and former Gadhafi regime members a serious threat to the countrys future.
Hundreds of Libyan tribal leaders have met in Cairo with Egyptian authorities hoping to enlist their help in preventing Islamist violence from spilling over their shared border.
According to a report in the Wall Street Journal Egypts military is supplying arms to Libyan rebels with the US governments full knowledge.
Despite NATO airstrike against convoy of Libyan military vehicles, rebels retreat east towards Ajdabiya after rocket, artillery fire
Penicillamine is a medicine available in a number of countries worldwide. A list of US medications equivalent to Penicillamine is available on the Drugs.com website.
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5-6 mm diameter, well-defined, hyperechoic solid lesion on the surface of the inferior aspect of the left testis. This could be either based on the tunica or i
That is a sweeping statement, if so he made Libyans the most economically prosperous people on the continent of Africa.(See GDP etc) Even his opponents do not deny the health care and educational provision his regime has created for the Libyans.. If you really want to talk about squandering oil revenues, you need look no further than the UK, when Thatcher reformed the British economy away from manufacturing into finance, banking and the markets and in the process created millions of unemployed whom she was quiet prepared to pauperism them in the long term by allowing them to live on unemployed benefits in the hope of taming them.. The legacy of which is still with us, i e a solid block of people who have hardly worked since, and who are insulted in the MSM as the underclass.. The Libyan people are in for a shock, by accepting the poisoned chalice of NATO they will live to regret it, as they will have no purchase on any incoming government who will be placed in office by western powers, ...
Walking along the tree-lined gravel track towards one of the Roman Empires greatest architectural legacies, little can prepare you for what you are about to experience.
Sani Iro, communications director for Nigers ruling party, says government forces are well equipped to prevent any security skirmishes
Originally Posted by iguanaman Maybe we dont enjoy helping terrorists as much as you. Your foreign policy indicates otherwise. Too bad theres not an
Expression of SLC3A1 (ATR1, CSNU1, D2H, NBAT, RBAT) in cervix, uterine tissue. Antibody staining with HPA038360 in immunohistochemistry.
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2 Makara, anul 413 al calendarului darian, vasul marțian de explorare Yossarian, orbită înaltă în jurul planetei Tau Ceti E (Genesis) „A. Examen extern (metodă vizuală și vizual-asistată) Cadavrul unui bărbat caucazian, vârstă între 40 și 45 de ani, constituție normală. 1. Semnele morții reale: lipsa pulsului ...
NIAMEY, Niger - Niger will not extradite Saadi Khadafy even though the son of the slain Libyan leader violated his asylum conditions with...
Originally Posted by Fenton This is starting to make allot of sense. A way to arm the Syrian rebels through a New mock Libyan Consulate. It would expl
The NATO chief has said he was confident that time was running out for Libyan leader Muammar Gaddafi, despite the prolonged stalemate between his forces and rebels who seek his ouster.
Rep. Darrell Issa dealt another blow to American security yesterday by compromising the identities of several Libyans working with the U.S. government. that
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Marine Drugs  | Free Full-Text | BMAA Inhibits Nitrogen Fixation in the Cyanobacterium Nostoc sp. PCC 7120 | HTMLMarine Drugs | Free Full-Text | BMAA Inhibits Nitrogen Fixation in the Cyanobacterium Nostoc sp. PCC 7120 | HTML

... and that the inhibition was considerably more severe than that induced by combined nitrogen sources and most other amino acids ... one being the neurotoxic non-protein amino acid β-N-methylamino-L-alanine (BMAA), proposed to be a causative agent of human ... Montesinos, M.L.; Herrero, A.; Flores, E. Amino acid transport systems required for diazotrophic growth in the cyanobacterium ... In contrast to the non-protein amino acid BMAA, all proteinogenic amino acids showed limited inhibitory effects on nitrogenase ...
more infohttp://www.mdpi.com/1660-3397/11/8/3091/htm

Amino acid transport System A resembles System N in sequence but differs in mechanism | PNASAmino acid transport System A resembles System N in sequence but differs in mechanism | PNAS

Classical amino acid transport System A accounts for most of the Na+-dependent neutral amino acid uptake by mammalian cells. ... unlike System ASC, System L does not depend on Na+. Thus, two of the three general amino acid transport systems mediate ... Amino acid transport System A resembles System N in sequence but differs in mechanism. Richard J. Reimer, Farrukh A. Chaudhry, ... Transport mediated by SA1 is also electrogenic. Amino acid transport Systems A and N thus appear closely related in function as ...
more infohttps://www.pnas.org/content/97/14/7715

Amino acid transport systems, neutral financial definition of Amino acid transport systems, neutralAmino acid transport systems, neutral financial definition of Amino acid transport systems, neutral

What is Amino acid transport systems, neutral? Meaning of Amino acid transport systems, neutral as a finance term. What does ... Amino acid transport systems, neutral mean in finance? ... Definition of Amino acid transport systems, neutral in the ... Amino acid transport systems, neutral financial definition of Amino acid transport systems, neutral https://financial- ... redirected from Amino acid transport systems, neutral). Also found in: Dictionary, Thesaurus, Medical, Legal, Encyclopedia. ...
more infohttps://financial-dictionary.thefreedictionary.com/Amino+acid+transport+systems%2C+neutral

The rBAT gene is responsible for L-cystine uptake via the b0,(+)-like amino acid transport system in a renal proximal tubular...The rBAT gene is responsible for L-cystine uptake via the b0,(+)-like amino acid transport system in a "renal proximal tubular"...

The rBAT gene is responsible for L-cystine uptake via the b0,(+)-like amino acid transport system in a "renal proximal tubular ... like amino acid transport system in a "renal proximal tubular" cell line (OK cells). Journal of Biological Chemistry, 271(18): ... We have also examined transport of l-cystine in OK cells and found characteristics very similar to the amino acid exchanger ... We have also examined transport of l-cystine in OK cells and found characteristics very similar to the amino acid exchanger ...
more infohttp://www.zora.uzh.ch/id/eprint/1364/

AID 456218 - Inhibition of amino acid transport system xc- in human SNB19 cells assessed as [3H]L-glutamate uptake at 500 uM by...AID 456218 - Inhibition of amino acid transport system xc- in human SNB19 cells assessed as [3H]L-glutamate uptake at 500 uM by...

Inhibition of amino acid transport system xc- in human SNB19 cells assessed as [3H]L-glutamate uptake at 500 uM by liquid ...
more infohttps://pubchem.ncbi.nlm.nih.gov/bioassay/456218

Renal transport of neutral amino acids. Tubular localization of Na+-dependent phenylalanine- and glucose-transport systems |...Renal transport of neutral amino acids. Tubular localization of Na+-dependent phenylalanine- and glucose-transport systems |...

Renal transport of neutral amino acids. Tubular localization of Na+-dependent phenylalanine- and glucose-transport systems. U ... Renal transport of neutral amino acids. Tubular localization of Na+-dependent phenylalanine- and glucose-transport systems ... Renal transport of neutral amino acids. Tubular localization of Na+-dependent phenylalanine- and glucose-transport systems ... Renal transport of neutral amino acids. Tubular localization of Na+-dependent phenylalanine- and glucose-transport systems ...
more infohttp://www.biochemj.org/content/220/1/15

Amino acid transport systems, neutral | definition of Amino acid transport systems, neutral by Medical dictionaryAmino acid transport systems, neutral | definition of Amino acid transport systems, neutral by Medical dictionary

Amino acid transport systems, neutral explanation free. What is Amino acid transport systems, neutral? Meaning of Amino acid ... transport systems, neutral medical term. What does Amino acid transport systems, neutral mean? ... Looking for online definition of Amino acid transport systems, neutral in the Medical Dictionary? ... Amino acid transport systems, neutral , definition of Amino acid transport systems, neutral by Medical dictionary https:// ...
more infohttps://medical-dictionary.thefreedictionary.com/Amino+acid+transport+systems%2C+neutral

Involvement of transporter recruitment as well as gene expression in the substrate-induced adaptive regulation of amino acid...Involvement of transporter recruitment as well as gene expression in the substrate-induced adaptive regulation of amino acid...

We investigated the molecular mechanism involved in the adaptive regulation of the amino acid transport system A, a process in ... N2 - We investigated the molecular mechanism involved in the adaptive regulation of the amino acid transport system A, a ... AB - We investigated the molecular mechanism involved in the adaptive regulation of the amino acid transport system A, a ... abstract = "We investigated the molecular mechanism involved in the adaptive regulation of the amino acid transport system A, a ...
more infohttps://augusta.pure.elsevier.com/en/publications/involvement-of-transporter-recruitment-as-well-as-gene-expression

xCT: Amino Acid Transport and Disorders of the Central Nervous System | Antibody News: Novus BiologicalsxCT: Amino Acid Transport and Disorders of the Central Nervous System | Antibody News: Novus Biologicals

... is a member of the heterodimeric amino acid transporter family. Proteins within this family are linked to one another via a ... xCT: Amino Acid Transport and Disorders of the Central Nervous System. Fri, 09/06/2013 - 13:53 ... Home » xCT: Amino Acid Transport and Disorders of the Central Nervous System ... an amino acid that is found only at very low levels in plasma and cerebrospinal fluid. The cysteine is subsequently transported ...
more infohttps://www.novusbio.com/antibody-news/antibodies/xct-amino-acid-transport-and-disorders-of-the-central-nervous-system

Recombinant Human xCT protein (ab112403) | AbcamRecombinant Human xCT protein (ab112403) | Abcam

Amino acid transport system xc xCT antibody. *Amino acid transport system xc- ... Belongs to the amino acid-polyamine-organocation (APC) superfamily. L-type amino acid transporter (LAT) (TC 2.A.3.8) family. ... Solute carrier family 7 (anionic amino acid transporter light chain, xc- system), member 11 ... Solute carrier family 7, (cationic amino acid transporter, y+ system) member 11 ...
more infohttps://www.abcam.com/Recombinant-Human-xCT-protein-ab112403.html

Metab, Misc + CDB Flashcards by Carla Marie Lee | BrainscapeMetab, Misc + CDB Flashcards by Carla Marie Lee | Brainscape

Sodium-dependent amino acid transport system in renal tubules and intestines 41 ... Branched-chain amino acids: leucine, isoleucine, valine (LIV) [Think MAPLE tree, tree of LIVe, has BRANCHes] ... Which among the branched chain amino acids has a higher plasma level than the rest of the accumulating substances ... D/O of amino acid metabolism that looks like tetanus (muscular rigidity + opisthotonus) ...
more infohttps://www.brainscape.com/flashcards/metab-misc-cdb-4026518/packs/5949532

The role of the neutral amino acid transporter SNAT2 in cell volume regulation.The role of the neutral amino acid transporter SNAT2 in cell volume regulation.

... accounts for the activity of transport system A for neutral amino acids in most mammalian tissues. As the transport process ... Sodium-dependent neutral amino acid transporter-2 (SNAT2), the ubiquitous member of SLC38 family, ... Amino Acid Transport System A / physiology*. Amino Acids, Neutral / metabolism*. Biological Transport. Cell Membrane / ... 0/Amino Acid Transport System A; 0/Amino Acids, Neutral; 0/Hypertonic Solutions; 0/RNA, Small Interfering; 0/SLC38A2 protein, ...
more infohttp://www.biomedsearch.com/nih/role-neutral-amino-acid-transporter/16734764.html

Inhibition of L-type amino acid transporter modulates the expression of cell cycle regulatory factors in KB oral cancer cells.Inhibition of L-type amino acid transporter modulates the expression of cell cycle regulatory factors in KB oral cancer cells.

... heptane-2-carboxylic acid (BCH), an inhibitor of L-type amino acid transporters, on the cell growth suppression in KB human ... 0/Amino Acid Transport Systems; 0/Cell Cycle Proteins From MEDLINE®/PubMed®, a database of the U.S. National Library of ... Amino Acid Transport Systems / antagonists & inhibitors*. Blotting, Western. Cell Cycle Proteins / physiology*. Cell Line, ... 20606299 - Inhibition of l-type amino acid transporter modulates the expression of cell cycle regu.... 8136159 - Cell cycle- ...
more infohttp://www.biomedsearch.com/nih/Inhibition-L-Type-Amino-Acid/20606299.html

Sequence Similarity 









- 4FB4: The Structure of an ABC-Transporter Family Protein from Rhodopseudomonas palustris in...Sequence Similarity - 4FB4: The Structure of an ABC-Transporter Family Protein from Rhodopseudomonas palustris in...

branched-chain amino acid transport system substrate-binding protein Aromatic compound transport protein 1076 ... Putative branched-chain amino acid transport system substrate-binding protein 1076 4 3UKJ 1 A Extracellular ligand-binding ... Putative branched-chain amino acid transport system substrate-binding protein 1076 7 4EYQ 1 A Extracellular ligand-binding ... Putative branched-chain amino acid transport system substrate-binding protein UNP residues 27-385 1076 ...
more infohttps://www.rcsb.org/pdb/explore/sequenceCluster.do?structureId=4FB4&entity=1&seqid=40

Frontiers | Aliskiren Administration during Early Postnatal Life Sex-Specifically Alleviates Hypertension Programmed by...Frontiers | Aliskiren Administration during Early Postnatal Life Sex-Specifically Alleviates Hypertension Programmed by...

... technology and renin-angiotensin system (RAS). Methods: Pregnant Sprague-Dawley rats received regular chow or chow supplemented ... technology and renin-angiotensin system (RAS). Methods: Pregnant Sprague-Dawley rats received regular chow or chow supplemented ... Pinto, V., Pinho, M.J., and Soares-da-Silva, P. (2013). Renal amino acid transport systems and essential hypertension. FASEB J. ... Slc6a19 gene encodes an amino acid transporter B0AT1. Slc4a4 gene encodes a sodium bicarbonate cotransporter (NBC) involved in ...
more infohttps://www.frontiersin.org/articles/10.3389/fphys.2016.00299/full

rbsC - Ribose ABC transporter permease - Paenibacillus polymyxa (strain E681) - rbsC gene & proteinrbsC - Ribose ABC transporter permease - Paenibacillus polymyxa (strain E681) - rbsC gene & protein

Branched-chain amino acid transport system / permease component family protein. Brevibacillus laterosporus GI-9 ... TransportSAAS annotation. ,p>Information which has been generated by the UniProtKB automatic annotation system, without manual ... Belongs to the binding-protein-dependent transport system permease family.SAAS annotation. Automatic assertion according to ...
more infohttps://www.uniprot.org/uniprot/E0R9T9

Veis 1087 - Inner-membrane translocator - Verminephrobacter eiseniae (strain EF01-2) - Veis 1087 gene & proteinVeis 1087 - Inner-membrane translocator - Verminephrobacter eiseniae (strain EF01-2) - Veis 1087 gene & protein

Branched-chain amino acid transport system / permease component family protein. BURCE. 345. ... TransportSAAS annotation. ,p>Information which has been generated by the UniProtKB automatic annotation system, without manual ... Belongs to the binding-protein-dependent transport system permease family.SAAS annotation. Automatic assertion according to ...
more infohttps://www.uniprot.org/uniprot/A1WGV6

Mediation of Highly Concentrative Uptake of Pregabalin by L-Type Amino Acid Transport in Chinese Hamster Ovary and Caco-2 Cells...Mediation of Highly Concentrative Uptake of Pregabalin by L-Type Amino Acid Transport in Chinese Hamster Ovary and Caco-2 Cells...

L-type amino acid transport system is an ubiquitous Na+-independent transport system for large, neutral amino acids across ... and system b0,+ is one of the Na+-independent amino acid transport systems in intestine (Costa et al., 2000). System b0,+ is ... Su TZ, Lunney E, Campbell G, and Oxender DL (1995) Transport of gabapentin, a gamma-amino acid drug, by system l alpha-amino ... 4; Table 1). This result strongly suggests that PGB and leucine share the same L-type amino acid transport system transport ...
more infohttp://jpet.aspetjournals.org/content/313/3/1406.long

Series Parallel Diagram Single Cell Analysis Of Kynurenine And System L Amino Acid Transport In T Cells Nature Communications ~...Series Parallel Diagram Single Cell Analysis Of Kynurenine And System L Amino Acid Transport In T Cells Nature Communications ~...

series parallel diagram single cell analysis of kynurenine and system l amino acid transport in t cells nature communications. ... Piezo Controllers Drivers For Nanopositioning Systems Series Parallel Diagram Position Control Electronics Optimizes System ...
more infohttps://tracologie.info/series-parallel-diagram

MEDLINE - Resultado p gina 1
	MEDLINE - Resultado p gina 1

0 (Amino Acid Transport Systems); 0 (Amino Acids, Aromatic); 0 (AroP protein, E coli); 0 (DNA, Bacterial); 0 (Escherichia coli ... methylotrophus AS1 mutants auxotrophic for aromatic amino acids. Thus, introduction of foreign amino acid transporter genes ... 919 amino acids [aa]), which puts MmeI also in subtype IIC of the restriction-modification systems. Based on a molecular model ... To improve the amino acid production by metabolic engineering, eliminating the pathway bottleneck is known to be very effective ...
more infohttp://bases.bireme.br/cgi-bin/wxislind.exe/iah/online/?IsisScript=iah/iah.xis&nextAction=lnk&base=MEDLINE&lang=p&format=standard.pft&indexSearch=EX&exprSearch=B03.440.400.425.525.520.500

Transporter expression and p-boronophenylalanine (BPA)  | Open-iTransporter expression and p-boronophenylalanine (BPA) | Open-i

Amino Acid Transport System y+/metabolism*. *Amino Acid Transport Systems/metabolism*. *Antigens, CD98 Light Chains/metabolism* ... Bottom Line: Among aromatic amino acid transporters, ATB(0,+), LAT1 and LAT2 were found to transport BPA with Km values of ... Bottom Line: Among aromatic amino acid transporters, ATB(0,+), LAT1 and LAT2 were found to transport BPA with Km values of ... Amino Acid Transport System y+/metabolism*. *Amino Acid Transport Systems/metabolism*. *Antigens, CD98 Light Chains/metabolism* ...
more infohttps://openi.nlm.nih.gov/detailedresult.php?img=PMC4376436_cas0106-0279-f5&req=4

Placental Metabolism and Regulation of Nutrient Transfer to the Fetus | Springer for Research & DevelopmentPlacental Metabolism and Regulation of Nutrient Transfer to the Fetus | Springer for Research & Development

Placental amino acid uptake. III. Transport systems for neutral amino acids. Am J Physiol. 1976;230:706-710.PubMedGoogle ... Transport of amino acids in the placenta. Biochim Biophys Acta. 1985;822:169-201.PubMedGoogle Scholar ... and amino acids; protein synthesis; amino acid interconversion; glycero-lipid synthesis; and chain lengthening or shortening of ... Umbilical uptake of amino acids in the unstressed fetal lamb. J Clin Invest. 1976;58:1428-1434.PubMedCrossRefGoogle Scholar ...
more infohttps://rd.springer.com/chapter/10.1007/978-1-4613-9240-8_3

PDF] Heteromeric amino acid transporters: biochemistry, genetics, and physiology. | Semantic ScholarPDF] Heteromeric amino acid transporters: biochemistry, genetics, and physiology. | Semantic Scholar

HATs represent several of the classic mammalian amino acid transport systems (e.g., L isoforms, y(+)L isoforms, asc, x(c)(-), ... In addition to the role in amino acid transport, one HSHAT [the heavy subunit of the cell-surface antigen 4F2 (also named CD98 ... The heteromeric amino acid transporters (HATs) are composed of two polypeptides: a heavy subunit (HSHAT) and a light subunit ( ... Obligatory amino acid exchange via systems bo,+-like and y+L-like. A tertiary active transport mechanism for renal reabsorption ...
more infohttps://www.semanticscholar.org/paper/Heteromeric-amino-acid-transporters%3A-biochemistry%2C-Chillar%C3%B3n-Roca/9d7fb82ef12d96940f9f0250932186321cae1e95

Frontiers | Threonine appears to be essential for proliferation of human as well as mouse embryonic stem cells | Cell and...Frontiers | Threonine appears to be essential for proliferation of human as well as mouse embryonic stem cells | Cell and...

Other essential and nonessential amino acids need not be added to the medium for mES cell proliferation. Furthermore, the ... cell proliferation depends exclusively on the nutritionally essential amino acid, L-threonine, in the medium. ... Care 17, 80-85.Abstract: Of the amino acids, threonine alone is essential for mouse embryonic stem (mES) cell proliferation ... Keywords: threonine, embryonic stem cells, cell proliferation, humans, amino acid transport systems ...
more infohttps://www.frontiersin.org/articles/10.3389/fcell.2014.00018/full

Essential Amino Acids
     Summary Report | CureHunterEssential Amino Acids Summary Report | CureHunter

Amino acids that are not synthesized by the human body in amounts sufficient to carry out physiological functions. They are ... Glycine (Aminoacetic Acid) 9. Amino Acid Transport Systems (Amino Acid Transporter) 10. Insulin (Novolin) ... Essential Amino Acids. Subscribe to New Research on Essential Amino Acids Amino acids that are not synthesized by the human ... Amino Acids, Peptides, and Proteins*Amino Acids: 10808*Essential Amino Acids: 403*Arginine: 7940 ...
more infohttp://www.curehunter.com/public/keywordSummaryD000601-Essential-Amino-Acids.do
  • In contrast, the chronic phase is associated with an induction of ata2 gene expression as evidenced from the increase in the steady-state levels of ATA2 mRNA, restoration of the intracellular pool of ATA2 protein, and blockade of the induction by cycloheximide and actinomycin D. The increase in system A activity induced by amino acid starvation is blocked specifically by system A substrates, including the non-metabolizable α-(methylamino)isobutyric acid. (elsevier.com)
  • As the transport process performed by SNAT2 is highly energized, system A substrates, such as glutamine, glycine, proline and alanine, reach high transmembrane gradients and constitute major components of the intracellular amino acid pool. (biomedsearch.com)
  • Uptake of PGB was mutually exclusive with leucine, GBP and 2-aminobicyclo(2,2,1)heptane-2-carboxylic acid, the substrates preferential for system L. The preloaded PGB in CHO cells was exchangeable with leucine, but at a lower exchange rate than that of leucine and GBP. (aspetjournals.org)
  • The different isoforms also exhibit different relative affinities for the amino acid substrates. (wikipedia.org)
  • Although molecular cloning has recently identified the proteins that mediate Systems ASC and L ( 13 - 17 ), System A has eluded biochemical purification, genetic approaches, and expression cloning in Xenopus oocytes ( 10 , 18 - 20 ). (pnas.org)
  • Under these conditions, the expression of SNAT2 gene is induced and newly synthesized SNAT2 proteins are preferentially targeted to the cell membrane, leading to a significant increase of system A transport Vmax. (biomedsearch.com)
  • The Amino Acid/Auxin Permease (AAAP) Family (TC# 2.A.18) is a family of secondary carrier proteins, a member of the APC Superfamily that includes hundreds of proteins from plants, animals, fungi and lower eukaryotes. (wikipedia.org)
  • The three main purposes of metabolism are the conversion of food/fuel to energy to run cellular processes, the conversion of food/fuel to building blocks for proteins, lipids, nucleic acids, and some carbohydrates, and the elimination of nitrogenous wastes. (wikipedia.org)
  • Metabolism is usually divided into two categories: catabolism, the breaking down of organic matter for example, the breaking down of glucose to pyruvate, by cellular respiration, and anabolism, the building up of components of cells such as proteins and nucleic acids. (wikipedia.org)
  • Proteins are made of amino acids arranged in a linear chain joined together by peptide bonds. (wikipedia.org)
  • Other proteins have structural or mechanical functions, such as those that form the cytoskeleton, a system of scaffolding that maintains the cell shape. (wikipedia.org)
  • Proteins are also important in cell signaling, immune responses, cell adhesion, active transport across membranes, and the cell cycle. (wikipedia.org)
  • Most of the structures that make up animals, plants and microbes are made from three basic classes of molecule: amino acids, carbohydrates and lipids (often called fats). (wikipedia.org)
  • Dr Julian Dow Laboratory of Cell Biology, Division of Cellular and Molecular Biology, Institute of Biomedical and Life Sciences, University of Glasgow: Genetic analysis of epithelial transport in Drosophila. (bio.net)
  • SNAT1 mediated L-citrulline transport modulates eNOS coupling and thus regulates NO production in hypoxic PAECs from newborn piglets. (gwu.edu)
  • The dual-component system, which consists of an integrative plasmid with a mini-Mu and an easily eliminated helper plasmid encoding inducible transposition factors, is described in detail as a tool for the integration/amplification of recombinant DNAs. (bireme.br)
  • The differential affinity of PGB and GBP at L-type system leads to more concentrative accumulation of PGB than GBP, which may facilitate PGB transmembrane absorption in vivo. (aspetjournals.org)
  • We have recently identified another member of this family as responsible for classical amino acid transport System N. Unlike the virtually ubiquitous Systems A, ASC, and L, System N appears more tissue-specific and mediates the uptake specifically of glutamine, histidine, and asparagine ( 25 ). (pnas.org)
  • Multiple hormones increase System A function acutely through changes in the driving force for transport as well as through longer-term changes in gene expression. (pnas.org)
  • Their function as exchangers presumably relies on the active accumulation of amino acids through other mechanisms. (pnas.org)
  • The transport properties for phenylalanine and glucose in luminal-membrane vesicles from outer cortex (pars convoluta) and outer medulla (pars recta) of rabbit kidney were studied by a spectrophotometric method. (biochemj.org)
  • Attempts to calculate the stoichiometry of the different Na+/D-glucose transport systems by using Hill-type plots revealed that the ratio of the Na+/hexose co-transport probably is 1:1 in the case of pars convoluta and 2:1 in membrane vesicles from pars recta. (biochemj.org)
  • These functionally defined systems were originally characterized by kinetic and competition studies ( Christensen, 1990 ). (aspetjournals.org)
  • Without comprehensive kinetic analysis, it is often difficult to specify a transport system responsible for uptake of an unknown substrate ( Christensen, 1990 ). (aspetjournals.org)
  • Poly(A)+ RNA from OK cells induced by system b0,(+)-like transport activity in oocytes. (uzh.ch)
  • The other two known system A subtypes, ATA1 and ATA3, are not expressed in these cells. (elsevier.com)
  • Exposure of these cells to an amino acid-free medium induces system A activity. (elsevier.com)
  • These results demonstrate the pivotal role played by SNAT2 induction in the short-term hypertonic RVI and suggest that neutral amino acids behave as compatible osmolytes in hypertonically stressed cells. (biomedsearch.com)
  • The word metabolism can also refer to the sum of all chemical reactions that occur in living organisms, including digestion and the transport of substances into and between different cells, in which case the set of reactions within the cells is called intermediary metabolism or intermediate metabolism. (wikipedia.org)