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  • intracellular
  • It is a rare autosomal recessive disorder resulting from accumulation of free cystine in lysosomes, eventually leading to intracellular crystal formation throughout the body. (wikipedia.org)
  • Vesicle mediated protein sorting plays an important role in segregation of intracellular molecules into distinct organelles. (antikoerper-online.de)
  • Regulated by various intracellular signaling pathways including inhibition by protein kinase A activation, and endogenously activation by the calmodulin complex, the calmodulin-dependent kinase II and LCK tyrosine kinase. (uniprot.org)
  • Such intracellular niches of various pathogens are diverse, and biogenesis often depends on the delivery of bacterial effector proteins into the host cell cytoplasm. (pubmedcentralcanada.ca)
  • Homologues of this family are found in various yeasts, plants, animals, archaea, and Gram-negative and Gram-positive bacteria termed "natural resistance-associated" macrophage proteins because one of the animal homologues plays a role in resistance to intracellular bacterial pathogens such as Salmonella enterica serovar Typhimurium, Leishmania donovani and Mycobacterium bovis. (wikipedia.org)
  • A third yeast protein, Smf3p, appears to be exclusively intracellular, possibly in the Golgi. (wikipedia.org)
  • Organism
  • p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence. (uniprot.org)
  • These include enzymes called ceramide-activated Ser-Thr phosphatases (CAPPs), such as protein phosphatase 1 and 2A (PP1 and PP2A), which were found to interact with ceramide in studies done in a controlled environment outside of a living organism (in vitro). (wikipedia.org)
  • solubility
  • As cystine is highly insoluble, when its concentration in tissue lysosomes increases, its solubility is immediately exceeded and crystalline precipitates are formed in almost all organs and tissues. (wikipedia.org)
  • Lipid
  • Lipid signaling, broadly defined, refers to any biological signaling event involving a lipid messenger that binds a protein target, such as a receptor, kinase or phosphatase, which in turn mediate the effects of these lipids on specific cellular responses. (wikipedia.org)
  • translocation
  • In general, the appearance of SMMs coincides with the onset of bacterial replication, and both phenomena are dependent on the translocation of effector proteins of the Salmonella Pathogenicity Island 2 (SPI2)-encoded type III secretion system (T3SS) ( 10 , 11 ). (pubmedcentralcanada.ca)
  • bound
  • Steroid hormones are generally carried in the blood, bound to specific carrier proteins such as sex hormone-binding globulin or corticosteroid-binding globulin. (wikipedia.org)
  • One study has found that these steroid-carrier complexes are bound by megalin, a membrane receptor, and are then taken into cells via endocytosis. (wikipedia.org)
  • Furthermore, a hydrophobic patch present in the domain also binds to a subset of D-phi-F/W motif-containing proteins that are bound by the alpha-adaptin appendage domain (epsin, AP180, eps15). (wikipedia.org)
  • There are three main proteins that the two hormones are bound to. (wikipedia.org)
  • Nuclear
  • In order to be active, steroid hormones must free themselves from their blood-solubilizing proteins and either bind to extracellular receptors, or passively cross the cell membrane and bind to nuclear receptors. (wikipedia.org)
  • Two types of unconventional protein secretion are these: signal-peptid-containing proteins and cytoplasmatic and nuclear proteins that are missing an ER-signal peptide (1). (wikipedia.org)
  • receptors
  • In their chemical communication, odorant-binding proteins and chemosensory proteins, which accumulate in the sensillum lymph in the antennae, play roles in transporting semiochemicals to chemosensory receptors. (pnas.org)
  • complexes
  • AP complexes connect cargo proteins and lipids to clathrin at vesicle budding sites, as well as binding accessory proteins that regulate coat assembly and disassembly (such as AP180, epsins and auxilin). (wikipedia.org)
  • Enzymes
  • On the other hand, studies in cells have shown that ceramide-inducing agents such as tumor necrosis factor-alpha α (TNFα) and palmitate induce the ceramide-dependent removal of a phosphate group (dephosphorylation) of the retinoblastoma gene product RB and the enzymes, protein kinases B (AKT protein family) and C α (PKB and PKCα). (wikipedia.org)
  • enzyme
  • Each of these mutations alters the gene's protein product (i.e., the enzyme), sometimes severely inhibiting its function. (wikipedia.org)
  • In molecular biology, autophagocytosis associated protein Atg3 is the E2 enzyme for the LC3 lipidation process. (wikipedia.org)
  • processes
  • These effector proteins manipulate a large number of host cell processes and force the host cell to create a suitable microenvironment for Salmonella ( 7 , 12 , 13 ). (pubmedcentralcanada.ca)
  • Most of these proteins are involved in processes in higher eukaryotes, however unconventional export mechanism was found in lower eukaryotes too. (wikipedia.org)
  • mutations
  • p>Describes annotations that are concluded from looking at variations or changes in a gene product such as mutations or abnormal levels and includes techniques such as knockouts, overexpression, anti-sense experiments and use of specific protein inhibitors. (uniprot.org)
  • Clathrin
  • Adaptins recognise and bind to clathrin through their hinge region (clathrin box), and recruit accessory proteins that modulate AP function through their C-terminal ear (appendage) domains. (wikipedia.org)
  • binds
  • p>This subsection of the 'Function' section describes a region in the protein which binds nucleotide phosphates. (uniprot.org)
  • Thyroid-stimulating hormone (TSH) released from the anterior pituitary gland binds the TSH receptor (a Gs protein-coupled receptor) on the basolateral membrane of the cell and stimulates the endocytosis of the colloid. (wikipedia.org)
  • receptor
  • The D-BP protein contains a peroxisomal targeting signal 1 (PTS1) unit at the C-terminus allowing for its transport into peroxisomes by the PTS1 receptor. (wikipedia.org)
  • transport
  • The exchange involves the active transport by a carrier protein called karyopherins. (wikipedia.org)
  • The generalized transport reaction catalyzed by NRAMP family proteins is: Me2+ (out) + H+ (out) ⇌ Me2+ (in) + H+ (in). (wikipedia.org)
  • The lipophilicity of T3 and T4 requires their binding to the protein carrier thyroid-binding protein (TBG) (thyroxine-binding globulins, thyroxine binding prealbumins, and albumins) for transport in the blood. (wikipedia.org)
  • Endocytosis is a form of active transport in which a cell transports molecules (such as proteins) into the cell (endo- + cytosis) by engulfing them in an energy-using process. (wikipedia.org)
  • These families are part of the Transport Classification (TC) system that is used by the International Union of Biochemistry and Molecular Biology (IUBMB) and are grouped according to characteristics such as the substrates being transported, the transport mechanism, the energy source used, and also by comparing the DNA sequences making up each protein. (wikipedia.org)
  • Examples of the P-type ATPase include Na+/K+-ATPase that is regulated by Janus Kinase-2 as well as Ca2+ ATPase which exhibits sensitivity to ADP and ATP concentrations P-glycoprotein is an example of an ABC transport binding protein in the human body. (wikipedia.org)
  • Disease
  • Tay-Sachs disease is an autosomal recessive genetic disorder, meaning that when both parents are carriers, there is a 25% risk of giving birth to an affected child with each pregnancy. (wikipedia.org)
  • commonly
  • section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein. (uniprot.org)
  • recessive
  • D-Bifunctional protein deficiency (officially called 17β-hydroxysteroid dehydrogenase IV deficiency) is an autosomal recessive peroxisomal fatty acid oxidation disorder. (wikipedia.org)
  • sequence
  • Note that the 'protein existence' evidence does not give information on the accuracy or correctness of the sequence(s) displayed. (uniprot.org)
  • interact
  • However, the number of these proteins is not sufficient to interact with a large number of semiochemicals. (pnas.org)
  • cell
  • The C-terminal domain of Beta2-adaptin is a protein domain is involved in cell trafficking by aiding import and export of substances in and out of the cell. (wikipedia.org)
  • The peroxisome is an organelle in the cell similar to the lysosome that functions to detoxify the cell. (wikipedia.org)
  • found
  • Orthologs of the TMCO6 protein have been found among sequenced organisms with the exception of invertebrates, fungi, plants and bacteria. (wikipedia.org)
  • disease
  • Prenatal testing is also available to determine if the fetus will have the disease or is a carrier. (wikipedia.org)
  • as published in Al-Bayan newspaper in 20 February 2016 making this family the largest one with patients and carriers of Danon disease. (wikipedia.org)
  • Tay-Sachs disease is an autosomal recessive genetic disorder, meaning that when both parents are carriers, there is a 25% risk of giving birth to an affected child with each pregnancy. (wikipedia.org)
  • amino acid
  • More recently, alterations in ketone body and amino acid/protein metabolism have been described during heart failure, as well as mitochondrial dysfunction and perturbed metabolic signaling (e.g., acetylation, O -GlcNAcylation). (onlinejacc.org)
  • Methionine is an essential amino acid required for protein synthesis and one-carbon metabolism. (wikipedia.org)
  • soluble
  • Utilizes liposomes and other carrier techniques to improve the solubility and delivery of taxol and other poorly-soluble anticancer agents. (buffalo.edu)
  • The association of the protein with membrane appeared to be loose as it could be easily recovered in soluble fraction. (jove.com)
  • MTR reactivation can also be NADPH dependent involving two redox proteins, soluble cytochrome b5 and reductase 1. (wikipedia.org)
  • yeast
  • A cell-free assay allows reconstitution of Vps33p-dependent transport to the yeast vacuole/lysosome. (nih.gov)
  • Intermediate
  • Methionine synthase reductase (MTRR) is primarily involved in the reductive methylation of homocysteine to methionine, utilizing methylcob(I)alamin as an intermediate methyl carrier. (wikipedia.org)
  • disorder
  • Sons of carriers have a 50 percent chance of inheriting the disorder. (wikipedia.org)
  • Affected men do not pass the disorder to their sons but their daughters will be carriers for the disorder. (wikipedia.org)
  • Prenatal testing is also available for known carriers of this disorder. (wikipedia.org)
  • Fructose malabsorption is a digestive disorder in which absorption of fructose is impaired by deficient fructose carriers in the small intestine's enterocytes. (wikipedia.org)
  • Molecular
  • With these techniques, we examine the effects of treatment upon tumor vascular permeability and drug deposition, the localization of the carrier-delivered drug within the tumor, and the molecular mechanisms involved when tumor blood vessels or tumor stroma are attacked during therapy. (buffalo.edu)
  • dependent
  • We documented that the stability of the protein is dependent on several key components of the secretion system and it has multiple interacting partners including a non-cag-PAI protein HP1489. (jove.com)
  • transports
  • Cystinosin functions as a symporter which actively transports protons and cystine, the oxidized cysteine dimer, out of the lysosome. (wikipedia.org)