IsoleucineBranched-chainProteinsZipperDehydrogenaseEnzymeKinasePathwayUptakeTransporterCofactorAmino acid transportSubstrateAccumulationMethionineIntracellularDorsalLeuTGlycineHydrophobicMitochondrialAcidRegulation of blood sugarVitroGlutamineIonsExtracellularHemoglobinGlucoseRegenerationMetabolismNuclearMutationEnzymesNutrientHydrolysisElectron transportMembranesCellFacilitateBindEssentialViralSpecificProcesses
Isoleucine7
- Maple syrup urine disease (MSUD), also known as branched-chain ketoaciduria, is an aminoacidopathy due to an enzyme defect in the catabolic pathway of the branched-chain amino acids leucine, isoleucine, and valine. (medscape.com)
- Maple syrup urine disease is caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase (BCKD) enzyme complex, which catalyses the decarboxylation of the alpha-keto acids of leucine, isoleucine, and valine to their respective branched-chain acyl-CoAs. (medscape.com)
- The three BCAAs are valine, leucine, and isoleucine, which have some important body functions. (medicinenet.com)
- The BCAAs are three essential amino acids - leucine, isoleucine, and valine. (medicinenet.com)
- These proteins provide all the essential amino acids, including isoleucine, leucine, and valine (the BCAAs). (medicinenet.com)
- Permease for leucine, valine and isoleucine. (ymdb.ca)
- Accumulation of leucine in particular causes neurological symptoms, whereas elevation of plasma isoleucine is associated with the maple syrup odor. (medscape.com)
Branched-chain1
- Diagnosis of maple syrup urine disease is by finding elevated plasma levels of branched-chain amino acids (particularly leucine) and confirmed by genetic testing. (msdmanuals.com)
Proteins7
- These specific transport proteins clear the synapse after a nerve signal, transporting neurotransmitters back into the axon and making it ready for another signal. (wikipedia.org)
- These proteins regulate selective transport, but exactly how they do this is still unclear. (rug.nl)
- These proteins are made up of domains comprising phenylalanine (F) and glycine (G) amino acids in tandem, and these play an essential role in transport. (rug.nl)
- The nucleoporins were tested for interactions with non-specific proteins and with chaperones, which are proteins that act as transport tickets through the pore. (rug.nl)
- This domain is the proposed leucine zipper at the N terminus of VPS13A and related proteins. (embl.de)
- This pattern is known to attract proteins that aid the cell in transporting other proteins inside its interior," says Meyer. (eurekalert.org)
- The proteins synthesized by parotid acinar cells are stored in large secretory granules whose composition includes α-amylase, leucine-rich parotid secretory protein (PSP), and proline-rich proteins (PRPs), in addition to multiple minor components 2-3 related to digestive and protective functions. (bvsalud.org)
Zipper3
- Leucines occurring in heptad repeats end up on the same sides of the helixes and are adjacent to each other in the stem of the Y (the "zipper" region). (lookformedical.com)
- A large superfamily of transcription factors that contain a region rich in BASIC AMINO ACID residues followed by a LEUCINE ZIPPER domain. (lookformedical.com)
- Here we report that a screening of an Arabidopsis transcription factor (TF) library using the homeodomain-leucine zipper I member AtHB23 as bait, allowed identifying the TF AtPHL1 interacting with the former. (gnu.ac.kr)
Dehydrogenase4
- Leucine dehydrogenase catalyzes the reversible oxidative deamination of L-LEUCINE, to 4-methyl-2-oxopentanoate (2-ketoisocaproate) and AMMONIA, with the corresponding reduction of the cofactor NAD+. (lookformedical.com)
- Overview: Leucine degradation in GapMind is based on MetaCyc pathway L-leucine degradation I, via branched alpha-keto acid dehydrogenase ( link ). (lbl.gov)
- The cofactor regeneration system of leucine dehydrogenase (LeuDH) and glucose dehydrogenase (GDH) has showed great coupling catalytic efficiency in the synthesis of l -tle, however the multi-enzyme complex of GDH and LeuDH has never been constructed successfully. (biomedcentral.com)
- Among those biocatalysts, leucine dehydrogenase (LeuDH, EC 1.4.1.9) exhibited an outstanding conversion efficiency and enantioselectivity which became the main method of l -tle synthesis in the market [ 11 ]. (biomedcentral.com)
Enzyme4
- A zinc containing enzyme of the hydrolase class that catalyzes the removal of the N-terminal amino acid from most L-peptides, particularly those with N-terminal leucine residues but not those with N-terminal lysine or arginine residues. (lookformedical.com)
- The activity of this enzyme is commonly assayed using a leucine arylamide chromogenic substrate such as leucyl beta-naphthylamide. (lookformedical.com)
- An enzyme that catalyzes the first step in the biosynthetic pathway to LEUCINE , forming isopropyl malate from acetyl-CoA and alpha-ketoisovaleric acid. (lookformedical.com)
- Peroxisomal biogenesis factor 7 also transports the enzyme phytanoyl-CoA hydroxylase (produced from the PHYH gene). (medlineplus.gov)
Kinase1
- Mutations in the catalytic Roc-COR and kinase domains of leucine-rich repeat kinase 2 (LRRK2) are a common cause of familial Parkinson's disease (PD). (nih.gov)
Pathway1
- The intracellular tranpsort of the leucine-labeled moiety of the glycoproteins follows the normal pathway. (rupress.org)
Uptake3
- The concentration difference creates an electrochemical potential gradient that is used to catalyze the uptake of organic substrates (in this case leucine). (wikipedia.org)
- The uptake of amino acids showed features characteristic of active transport. (microbiologyresearch.org)
- After correction for a non-mediated transport component, the kinetics of glutamate, glutamine, alanine and aspartate uptake measured over a wide concentration range followed the simple Michaelis-Menten saturation curves. (microbiologyresearch.org)
Transporter7
- Bacterial Leucine Transporter (LeuT) is a bundled twelve alpha helix protein which belongs to the family of transporters that shuttle amino acids in and out of bacterial cells. (wikipedia.org)
- Specialized in small hydrophobic amino acids such as leucine and alanine, this transporter is powered by the gradient of sodium ions that is normally maintained by healthy cells across their membranes. (wikipedia.org)
- Bacterial Leucine Transporter imports leucine from the outside of the cell to the inside with the aid of two sodium ions. (wikipedia.org)
- Leucine transporter, like the neurotransmitter transporters in nerve cells, has a bundle of twelve alpha helices that form a transport channel through the membrane. (wikipedia.org)
- As an ABC Transporter , ABCG2 exhibits ATPase activity and uses the energy of ATP hydrolysis to facilitate transport. (proteopedia.org)
- One molecule of ATP is hydrolyzed to transport substrates across the cell membrane while the second molecule of ATP is hydrolyzed to reset the transporter to its inward-facing conformation. (proteopedia.org)
- The overall shift from inward-facing to outward-facing promotes the transport of substrates through the transporter. (proteopedia.org)
Cofactor1
- Biosynthesis of l - tert -leucine ( l -tle), a significant pharmaceutical intermediate, by a cofactor regeneration system friendly and efficiently is a worthful goal all the time. (biomedcentral.com)
Amino acid transport1
- Defects of amino acid transport in the renal tubule include cystinuria and Hartnup disease, which are discussed elsewhere. (msdmanuals.com)
Substrate1
- The protein is in the inward-facing conformation with Cavity 1 open to the cytosol for substrate recruitment, the Leucine Plug intact, and Cavity 2 completely occluded. (proteopedia.org)
Accumulation1
- Accumulation of plasma leucine causes neurological symptoms. (medscape.com)
Methionine1
- Methionine transport by mycelia of Fusarium oxysporum f. sp. (microbiologyresearch.org)
Intracellular2
- Some genetic changes even affect intracellular protein transport through this process. (eurekalert.org)
- The intracellular transport of glycoproteins pulse-labeled in vitro with tritiated leucine and galactose in the surface mucous lining cells (SMC) of the fundus of the rat stomach was studied by electron microscope autoradiography. (rupress.org)
Dorsal1
- Lasek R. Axoplasmic transport in cat dorsal root ganglion cells: as studied with [3-H]-L-leucine. (harvard.edu)
LeuT3
- LeuT acts as a symporter, which means that it links the passage of a sodium ion across the cell membrane with the transport of the amino acid in the same direction. (wikipedia.org)
- As LeuT is a symporter and uses the electrochemical potential of sodium ions to facilitate leucine's transport, both sodium ions and the hydrophobic amino acid, Leucine (Leu), bind to the centre of this protein. (wikipedia.org)
- LeuT acts as a symporter, an integral membrane protein that works as a cotransporter linking the passage of two sodium ions across the membrane with the transport of the amino acid in the same direction thus making the process energetically favorable. (wikipedia.org)
Glycine2
- In addition to the FG repeats, some nucleoporins also contain domains of glycine, leucine, phenylalanine and glycine, or GLFG repeats. (rug.nl)
- This protein is based on the average properties of a class of Nucleoporins rich in the amino acid motif Glycine-Leucine-Phenylalanine-Glycine. (rug.nl)
Hydrophobic1
- Leucine and alanine are the main amino acids that this protein brings across the membrane since it has a high affinity for these small hydrophobic molecules. (wikipedia.org)
Mitochondrial2
- In a screen for mutants with abnormal mitochondrial morphology, we identified MMA-1, the Caenorhabditis elegans homolog of the French Canadian Leigh Syndrome protein LRPPRC (leucine-rich pentatricopeptide repeat containing). (cipsm.de)
- Our data reveal an evolutionary conserved mechanism that is triggered by reduced complex IV function and that induces mitochondrial hyperfusion to transiently compensate for a drop in the activity of the electron transport chain. (cipsm.de)
Acid2
- Leucine is an amino acid commonly found as a component of total parenteral nutrition. (drugbank.com)
- The most common mutation responsible for RCDP1 replaces the amino acid leucine at protein position 292 with a premature stop signal in the instructions for making peroxisomal biogenesis factor 7 (written as Leu292Ter or L292X). (medlineplus.gov)
Regulation of blood sugar1
- Claim) Leucine helps with the regulation of blood-sugar levels, the growth and repair of muscle tissue (such as bones, skin and muscles), growth hormone production, wound healing as well as energy regulation. (drugbank.com)
Vitro1
- A detailed modeling & simulation program for in vitro metabolism & transport studies. (simulations-plus.com)
Glutamine1
- Leucine is rapidly transported across the blood-brain barrier and is metabolized to presumably yield glutamate and glutamine. (medscape.com)
Ions2
- The transport is powered by the gradient of sodium ions, which generates a difference of electric potential between the inner and extracellular space. (wikipedia.org)
- It opens toward the extracellular space so that leucine and sodium ions can enter and bind to it. (wikipedia.org)
Extracellular1
- The TMD is responsible for binding and transporting substrates, is embedded in the cell membrane, extends into the extracellular region (Figure 1). (proteopedia.org)
Hemoglobin1
- Hemoglobin , another protein, transports oxygen to every cell. (medicinenet.com)
Glucose1
- They cause the Glut1 protein to lose its function in the cell membrane: the protein no longer transports glucose from the blood into the brain. (eurekalert.org)
Regeneration1
- A conditioning lesion induces changes in gene expression and axonal transport that enhance regeneration by increasing the intrinsic growth state of axons. (harvard.edu)
Metabolism1
- A number of endogenous systems, such as the aerobic metabolism and electron transport chains, generate highly reactive molecules with important biological functions known as reactive oxygen species (ROS), including superoxide and hydrogen peroxide (H2O2). (bvsalud.org)
Nuclear1
- Rev interacts with Crm1 through a leucine-rich nuclear export signal located in its C-terminus, and with RRE through an Arginine rich motif (ARM) located in the N-terminal region. (nih.gov)
Mutation1
- An example is a mutation in the gene for the Glut1 protein that causes two specific building blocks of protein, namely leucines, to lie next to one another, creating a so-called dileucine motif. (eurekalert.org)
Enzymes2
- Peroxisomal biogenesis factor 7 transports several enzymes that are essential for the normal assembly and function of peroxisomes. (medlineplus.gov)
- The PEX7 gene mutations responsible for RCDP1 prevent peroxisomal biogenesis factor 7 from transporting critical enzymes, particularly alkylglycerone phosphate synthase, into peroxisomes. (medlineplus.gov)
Nutrient1
- Potentiates nutrient transport into muscle. (aaasups.co.uk)
Hydrolysis1
- M17 LAP (Leucine Amino Peptidase) plays an important role in the hydrolysis of amino acids essential for growth and development of Plasmodium vivax (Pv), the pathogen causing malaria. (simulations-plus.com)
Electron transport1
- Reducing mma-1/ LRPPRC function also decreases the activity of complex IV of the electron transport chain, however without affecting cellular ATP levels. (cipsm.de)
Membranes2
- The cellular apparatus involved in pinching off vesicles from the cell membranes and transporting them into the cell's interior via endocytosis is partially responsible for misrouting the Glut1 protein. (eurekalert.org)
- Dr. Eren received her Ph.D. from Worcester Polytechnic Institute (WPI), where she studied metal transport mechanisms across membranes by P-type ATPases. (nih.gov)
Cell2
- In the cell, large molecules that must be transported into or out of the nucleus can only do so when they are attached to such a chaperone. (rug.nl)
- Furthermore, the recognition of Golgi-derived microtubules and their specific functions, the better understanding of Golgi transport processes, the recognition of the Golgi apparatus as a sensor of cellular stress and as trigger of Golgi-specific cell death pathways provide new hints to the molecular mechanisms underlying Golgi pathology. (frontiersin.org)
Facilitate1
- This demonstrated that the NupX pores are fully functional: they are able to facilitate selective transport. (rug.nl)
Bind1
- Antidepressants such as selective serotonin reuptake inhibitors (SSRIs) bind in the large cavity at the outer entrance of the protein, not directly by competitively inhibiting the binding site for leucine, but presumably blocking the motion of the protein that is necessary for undergoing the conformational change that leads to the release of leucine. (wikipedia.org)
Essential1
- We previously studied the pores in all their complexity, but for this study, we created a drastically simplified 'designer' pore to investigate the essential physical mechanisms of transport. (rug.nl)
Viral1
- the swabs were placed in sterile tubes containing 2 mL of viral transport media. (cdc.gov)
Specific1
- A transfer RNA which is specific for carrying leucine to sites on the ribosomes in preparation for protein synthesis. (lookformedical.com)
Processes1
- have been shown to affect Golgi structure or transport processes to and from the Golgi. (frontiersin.org)