The mitochondrial unfolded protein response (UPRmt) is turned on in response to mitochondrial stress. Mitochondrial stress ... Mitochondrial DNA has been known to encode 13 proteins. Recently, other short protein coding sequences have been identified, ... Mitochondrial DNA is a DAMP, which only becomes available during mitochondrial damage. Blood mitochondrial DNA levels become ... The mitochondrial free radical theory of ageing (MFRTA) proposes that free radicals produced by mitochondrial activity damage ...

These proteins are located on the inner mitochondrial membrane. Israel reconstituted this system using proteins he purified, ... he first determined the molar stoichiometry of the mitochondrial P450 system proteins using specific antibodies that he ... During his Ph.D. thesis research, Israel isolated the mitochondrial enzymes that catalyze the first step in the synthesis of ... By computerized analysis of amino acid sequences he predicted that the central rod domain of intermediate filament proteins is ...

Adrenodoxin functions as an electron transfer protein in the mitochondrial cytochrome P450 systems. The first enzyme in this ... Adrenal ferredoxin (also adrenodoxin (ADX), adrenodoxin, mitochondrial, hepatoredoxin, ferredoxin-1 (FDX1)) is a protein that ... Araya Z, Hosseinpour F, Bodin K, Wikvall K (2003). "Metabolism of 25-hydroxyvitamin D3 by microsomal and mitochondrial vitamin ... "Entrez Gene: FDX1 ferredoxin 1". Hanukoglu I, Jefcoate CR (Apr 1980). "Mitochondrial cytochrome P-450scc. Mechanism of electron ...

"NDUFAF7 - Protein arginine methyltransferase NDUFAF7, mitochondrial precursor - Homo sapiens (Human) - NDUFAF7 gene & protein ... Protein arginine methyltransferase NDUFAF7, mitochondrial, also known as NADH:ubiquinone oxidoreductase complex assembly factor ... In addition to co-complexes, NDUFAF4 has protein-protein interactions with LRRK2, q5nfq6_fratt, and q5ngw2_fratt. GRCm38: ... "Cardiac Organellar Protein Atlas Knowledgebase (COPaKB) -- Protein Information". amino.heartproteome.org. Retrieved 2018-07-27 ...

1999). "Heparin-binding protein targeted to mitochondrial compartments protects endothelial cells from apoptosis". J. Clin. ... Azurocidin also known as cationic antimicrobial protein CAP37 or heparin-binding protein (HBP) is a protein that in humans is ... The protein encoded by this gene is an azurophil granule antimicrobial protein, with monocyte chemotactic and antibacterial ... This encoded protein is a member of the PA clan of proteases but it is not a serine proteinase, because the active site serine ...

"Inhibitor of apoptosis proteins are substrates for the mitochondrial serine protease Omi/HtrA2". The Journal of Biological ... HtrA2 peptidase (EC 3.4.21.108, high temperature requirement protein A2, HtrA2, Omi stress-regulated endoprotease, serine ... Protein Expression and Purification. 19 (2): 227-34. doi:10.1006/prep.2000.1240. PMID 10873535. Martins LM, Turk BE, Cowling V ... "Structural insights into the pro-apoptotic function of mitochondrial serine protease HtrA2/Omi". Nature Structural Biology. 9 ( ...

Heat shock protein 75 kDa, mitochondrial is a protein that in humans is encoded by the TRAP1 gene. TRAP1 has been shown to ... "The hsp90-related protein TRAP1 is a mitochondrial protein with distinct functional properties". J Biol Chem. 275 (5): 3305-12 ... "Entrez Gene: TRAP1 TNF receptor-associated protein 1". Simmons, A D; Musy M M; Lopes C S; Hwang L Y; Yang Y P; Lovett M ( ... Song HY, Dunbar JD, Zhang YX, Guo D, Donner DB (March 1995). "Identification of a protein with homology to hsp90 that binds the ...

Transport of PEP across the mitochondrial membrane is accomplished by dedicated transport proteins; however no such proteins ... it triggers phosphorylation of enzymes and regulatory proteins by Protein Kinase A (a cyclic AMP regulated kinase) resulting in ... In the liver, the FOX protein FOXO6 normally promotes gluconeogenesis in the fasted state, but insulin blocks FOXO6 upon ... The majority of the enzymes responsible for gluconeogenesis are found in the cytosol; the exceptions are mitochondrial pyruvate ...

By focusing on CYC1, the gene encoding one form of the yeast mitochondrial protein cytochrome c, he was able to answer many ... Understanding the roles of various mitochondrial proteins in bioenergetics and metabolic processes; and Batten disease ( ... Identification of mutations affecting protein folding and stability; 7) Detection of the effects of chaperones on protein ... A key finding was that AUG was the primary initiation codon in eukaryotic protein translation. The deduction of the CYC1 DNA ...

The protein contains a mitochondrial signal peptide localizing it to the mitochondrial matrix. Analysis via the EXPASY ... TCAIM is a protein that in humans is encoded by the TCAIM gene (T-cell activation inhibitor, mitochondrial). The gene is ... The high isoelectric point of the Human protein provides further evidence for the mitochondrial localization due to the high pH ... 2001). "Toward a Catalog of Human Genes and Proteins: Sequencing and Analysis of 500 Novel Complete Protein Coding Human cDNAs ...

"Notch-activated signaling cascade interacts with mitochondrial remodeling proteins to regulate cell survival". Proceedings of ...

... processes the nuclear encoded protein DIABLO. The gene IMMP2L encodes protein inner mitochondrial membrane peptidase ... This protein catalyzes the removal of transit peptides required for the targeting of proteins from the mitochondrial matrix, ... this protein catalyzes the removal of transit peptides required for the targeting of proteins from the mitochondrial matrix, ... The human protein inner mitochondrial membrane peptidase subunit 2 has two isoforms due to alternative splicing. One isoform is ...

28S ribosomal protein S17, mitochondrial is a protein that in humans is encoded by the MRPS17 gene. Mammalian mitochondrial ... The encoded protein is moderately conserved between human mitochondrial and prokaryotic ribosomal proteins. Pseudogenes ... "Entrez Gene: MRPS17 mitochondrial ribosomal protein S17". Mao M, Fu G, Wu JS, et al. (1998). "Identification of genes expressed ... 2001). "The human mitochondrial ribosomal protein genes: mapping of 54 genes to the chromosomes and implications for human ...

Elongation factor Tu, mitochondrial is a protein that in humans is encoded by the TUFM gene. It is an EF-Tu homolog. GRCh38: ... Woriax VL, Burkhart W, Spremulli LL (1996). "Cloning, sequence analysis and expression of mammalian mitochondrial protein ... 2007). "Large-scale mapping of human protein-protein interactions by mass spectrometry". Mol. Syst. Biol. 3 (1): 89. doi: ... 2003). "A proteomics strategy to elucidate functional protein-protein interactions applied to EGF signaling". Nat. Biotechnol. ...

As a mitochondrial protein, it is involved in maintaining membrane potential and mitochondrial DNA (mtDNA) integrity, as well ... The mitochondrial targeting sequence at its N-terminal directs the localization of the protein to the mitochondrial matrix. ... This protein belongs to the DNAJ/Hsp40 protein family, which is known for binding and activating Hsp70 chaperone proteins to ... where it can also interact with cytosolic proteins and possibly function to transport these proteins. This protein is ...

Mitochondrial complex III deficiency nuclear type 6 is autosomal recessive. "CYC1 - Cytochrome c1, heme protein, mitochondrial ... which accepts electrons from Rieske protein and transfers electrons to cytochrome c in the mitochondrial respiratory chain. It ... Protein pages needing a picture, Genes on human chromosome 8, Transmembrane proteins, Cellular respiration). ... As an iron-sulfur protein approaches the b-c1 complex, it accepts an electron from the cytochrome b subunit, then undergoes a ...

"Ceramide Channels Increase the Permeability of the Mitochondrial Outer Membrane to Small Proteins". J. Biol. Chem. 277 (30): ... Ceramide has also been shown to form organized large channels traversing the mitochondrial outer membrane. This leads to the ... Kogot-Levin A, Saada A (2014). "Ceramide and the mitochondrial respiratory chain". Biochimie. 100: 88-94. doi:10.1016/j.biochi. ... Ceramide is subsequently transported to the Golgi apparatus by either vesicular trafficking or the ceramide transfer protein ...

NAD(P) transhydrogenase, mitochondrial is an integral protein of the inner mitochondrial membrane. The enzyme couples hydride ... The protein comprises three subunits (dI, dII and dIII), with the dII component spanning the inner mitochondrial membrane. X- ... "Toward a catalog of human genes and proteins: sequencing and analysis of 500 novel complete protein coding human cDNAs". Genome ... NAD(P) transhydrogenase, mitochondrial is an enzyme that in humans is encoded by the NNT gene on chromosome 5. The NNT gene ...

Portal: Biology (Genes on human chromosome 11, EC 2.3.1, Mitochondrial proteins, Glycolysis). ... Of the mitochondrial antigens that can generate autoantibody reactivity in PBC patients, the E2 subunit of the pyruvate ... These are called anti-mitochondrial antibodies (AMA) and anti-nuclear antibodies (ANA), respectively. These antibodies are ... Protein Structure and Molecular Enzymology. 1385 (2): 353-66. doi:10.1016/S0167-4838(98)00079-X. PMID 9655933. Hanemaaijer R, ...

Uncoupling protein 1 (UCP1), a unique brown fat mitochondrial protein, is also positive. There are structural rearrangements of ... with hibernoma Oil Red O-positive droplets of cytoplasmic lipid can be seen in most cases The neoplastic cells are S100 protein ...

... and 134-458 is the mature protein Serine protease HTRA2, mitochondrial, and its theoretical pI of this protein is 6.12. HtrA2 ... Serine protease HTRA2, mitochondrial is an enzyme that in humans is encoded by the HTRA2 gene. This protein is involved in ... The human protein Serine protease HTRA2, mitochondrial is 49kDa in size and composed of 458 amino acids. The peptide fragment ... "Inhibitor of apoptosis proteins are substrates for the mitochondrial serine protease Omi/HtrA2". The Journal of Biological ...

... a metallo-β-lactamase protein, as a human mitochondrial endoribonuclease". Nucleic Acids Research. 44 (4): 1813-32. doi:10.1093 ... and snRNA molecules LACTB2 is a mitochondrial endoribonuclease which may have a role in degrading mitochondrial mRNAs. A tumor- ... The LACTB2 protein has a metallo β-lactamase (MBL) fold, with two zinc ions in the active site. The metallo beta-lactamases ... Lactamase, beta 2 is a protein that in humans is encoded by the LACTB2 gene. LACTB2 is located on the 8th chromosome, with its ...

"Acinetobacter baumannii outer membrane protein a induces dendritic cell death through mitochondrial targeting". The Journal of ... Outer membrane protein A (OmpA) has been shown to be involved in the adherence of A. baumannii to epithelial cells. This allows ... Efflux pumps are protein machines that use energy to pump antibiotics and other small molecules that get into the bacterial ... The protein was also shown to localize to the mitochondria of epithelial cells and cause necrosis by stimulating the production ...

... also known as TPR repeat protein 19 or Tetratricopeptide repeat protein 19, mitochondrial is a protein that in humans is ... Rieske protein), UQCRB, "14kDa protein", UQCRH (cyt c1 Hinge protein), Rieske Protein presequence, "cyt. c1 associated protein ... This protein is embedded in the inner mitochondrial membrane and is involved in the formation of the mitochondrial respiratory ... "TTC19 - Tetratricopeptide repeat protein 19, mitochondrial precursor - Homo sapiens (Human) - TTC19 gene & protein". www. ...

"A subfamily of stress proteins facilitates translocation of secretory and mitochondrial precursor polypeptides". Nature. 332 ( ... The Sec proteins are strikingly conserved and the trafficking mechanisms that Schekman discovered are at the heart of ... Schekman, R.; Orci, L. (1996). "Coat Proteins and Vesicle Budding". Science. 271 (5255): 1526-1533. Bibcode:1996Sci...271.1526S ...

... mitochondrial is a protein that in humans is encoded by the ABCB6 gene. The membrane-associated protein encoded by this gene is ... 2000). "MTABC3, a novel mitochondrial ATP-binding cassette protein involved in iron homeostasis". J. Biol. Chem. 275 (23): ... ABCB6+protein,+human at the U.S. National Library of Medicine Medical Subject Headings (MeSH) ABCB6 human gene location in the ... The protein also carries the Lan antigen, which defines the Lan blood group system. ATP-binding cassette transporter GRCh38: ...

... in enabling the post-translational insertion of proteins into the endoplasmic reticulum and mitochondrial membranes. This was ... to tether cellular proteins to a ubiquitin ligase, resulting in ubiquitination and degradation of the tethered protein. This ... A subfamily of stress proteins facilitates translocation of secretory and mitochondrial precursor polypeptides. Nature 332, 800 ... Protein translocation mutants defective in the insertion of integral membrane proteins into the endoplasmic reticulum. Mol. ...

Lionaki E, Gkikas I, Daskalaki I, Ioannidi MK, Klapa MI, Tavernarakis N (2022). "Mitochondrial protein import determines ... "Mitochondrial protein import determines lifespan through metabolic reprogramming and de novo serine biosynthesis". Nature ... by which diverse physiological signals are integrated to modulate cellular mitochondrial content, protein synthesis, and energy ... implicated in regulating targeted protein turnover in stomatins and other membrane-associated proteins". Trends in Biochemical ...

October 2004). "Essential role of Mia40 in import and assembly of mitochondrial intermembrane space proteins". The EMBO Journal ... Following import of small Tim proteins into the intermembrane space Mia40 interacts with small Tim proteins via disulfide bonds ... Small Tim proteins are then maintained in active conformation within the intermembrane space by Hot13 (helper of Tims). It is ... MIA is composed of two main essential cysteine-rich proteins; Mia40 and Erv1. Mia40 is also referred to as Tim40 in yeast and ...

"A subfamily of stress proteins facilitates translocation of secretory and mitochondrial precursor polypeptides". Nature. 332 ( ... Inner and Outer Envelope Membrane Proteins by Two-Dimensional Gel Electrophoresis and Their Comparison with Stromal Proteins". ... Werner-Washburne was part of a team that discovered that a group of heat-shock proteins were chaperones. Werner-Washburne began ...