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Mitochondrial uncoupling protein 3 (UCP3) is a members of the larger family of mitochondrial anion carrier proteins (MACP). UCPs facilitate the transfer of anions from the inner to the outer mitochondrial membrane and transfer of protons from the outer to the inner mitochondrial membrane, reducing the mitochondrial membrane potential in mammalian cells.
DAP3 is a 28S subunit protein of mitoribosomes and localizes to the mitochondrial matrix. [7] [8] [9] As part of the mitoribosome, DAP3 participates in the translation of the 13 ETC complex proteins encoded in the mitochondrial genome, and consequently, in the regulation of cellular respiration.
NAD-dependent deacetylase sirtuin-3, mitochondrial also known as SIRT3 is a protein that in humans is encoded by the SIRT3 gene [sirtuin (silent mating type information regulation 2 homolog) 3 (S. cerevisiae)]. [5] [6] SIRT3 is member of the mammalian sirtuin family of proteins, which are homologs to the yeast Sir2 protein.
MT-ND3 is located in human mitochondrial DNA from base pair 10,059 to 10,404. [5] The MT-ND3 gene produces a 13 kDa protein composed of 115 amino acids. [9] [10] MT-ND3 is one of seven mitochondrial genes encoding subunits of the enzyme NADH dehydrogenase (ubiquinone), together with MT-ND1, MT-ND2, MT-ND4, MT-ND4L, MT-ND5, and MT-ND6.
Mitochondrial ribosomal protein L3 is a protein that in humans is encoded by the MRPL3 gene. [5] Mammalian mitochondrial ribosomal proteins are encoded by nuclear genes and help in protein synthesis within the mitochondrion. Mitochondrial ribosomes (mitoribosomes) consist of a small 28S subunit and a large 39S subunit.
The dimeric mitochondrial protein encoded by this gene is known to induce apoptosis, even in the presence of BCL2. [9] Change of BNIP3 expression along other members of the Bcl-2 family measured by qPCR captures important characteristics of malignant transformation, and are defined as markers of resistance toward cell death, a key hallmark of ...
[8] [9] It is a potent protein disaggregase that chaperones the mitochondrial intermembrane space. [10] Mutations in the CLPB gene could cause autosomal recessive metabolic disorder with intellectual disability/developmental delay, congenital neutropenia, progressive brain atrophy, movement disorder, cataracts, and 3-methylglutaconic aciduria.
The GTPBP3 gene contains 10 exons, [6] and encodes a ~44 kDa GTP-binding protein that is evolutionarily conserved from bacteria to mammals. [7] The N-terminal domain of mitochondrial tRNA modification GTPase mediates the dimerization of the protein in a potassium-independent manner, [8] which is thought to be related to the construction of the binding site for the one-carbon-unit donor in its ...
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