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*  Mitochondrial DNA
The replisome machinery is formed by DNA polymerase, TWINKLE and mitochondrial SSB proteins. TWINKLE is a helicase, which ... Haplogroup Heteroplasmy Human mitochondrial DNA haplogroup Human mitochondrial genetics Mitochondrial disease Mitochondrial DNA ... Between most (but not all) protein-coding regions, tRNAs are present (see the human mitochondrial genome map). During ... Mutations in nuclear genes that encode proteins that mitochondria use can also contribute to mitochondrial diseases. These ...
*  Mitochondrial protein-transporting ATPase
... mitochondrial protein-importing). During the interactions of the proteins Hsp70 and sunbit Tim44, ATPase domain is essential. ... In enzymology, a mitochondrial protein-transporting ATPase (EC 3.6.3.51) is an enzyme that catalyzes the chemical reaction ATP ... Krimmer, T.; Rassow, J.; Kunau, W. H.; Voos, W.; Pfanner, N. (2000-08-01). "Mitochondrial protein import motor: the ATPase ... Moro, Fernando; Okamoto, Koji; Donzeau, Mariel; Neupert, Walter; Brunner, Michael (2002-03-01). "Mitochondrial protein import: ...
*  Mitochondrial trifunctional protein
... (MTP) is a protein attached to the inner mitochondrial membrane which catalyzes three out ... mitochondrial trifunctional protein TP at the US National Library of Medicine Medical Subject Headings (MeSH). ... identified a human mitochondrial protein, monolysocardiolipin acyltransferase (MLCL AT-1), that is identical in amino acid ... Zhou, Z.; Zhou, J.; Du, Y. (2012). "Estrogen Receptor Alpha Interacts with Mitochondrial Protein HADHB and Affects Beta- ...
*  Mitochondrial unfolded protein response
Unfolded protein response Pellegrino MW, Nargund AM, Haynes CM (2013). "Signaling the mitochondrial unfolded protein response ... The mitochondrial unfolded protein response (UPRmt) is a cellular stress response related to the mitochondria. The UPRmt ... Papa L, Germain D (2014). "SirT3 regulates the mitochondrial unfolded protein response". Molecular and Cellular Biology. 34 (4 ... The UPRmt can occur either in the mitochondrial matrix or in the mitochondrial inner membrane. In the UPRmt, the mitochondrion ...
*  Mitochondrial ribosomal protein l13
... is a protein that in humans is encoded by the MRPL13 gene. Mammalian mitochondrial ... ribosomal proteins are encoded by nuclear genes and help in protein synthesis within the mitochondrion. Mitochondrial ribosomes ... Mitochondrial ribosomal protein L13". Retrieved 2017-07-05. Mukhopadhyay R, Ray PS, Arif A, Brady AK, Kinter M, Fox PL (2008 ... They have an estimated 75% protein to rRNA composition compared to prokaryotic ribosomes, where this ratio is reversed. Another ...
*  Mitochondrial ribosomal protein L41
39S ribosomal protein L41, mitochondrial is a protein that in humans is encoded by the MRPL41 gene. Mammalian mitochondrial ... 2005). "Mitochondrial ribosomal protein L41 suppresses cell growth in association with p53 and p27Kip1". Mol. Cell. Biol. 25 ( ... 2006). "Mitochondrial ribosomal protein L41 mediates serum starvation-induced cell-cycle arrest through an increase of p21(WAF1 ... "Entrez Gene: MRPL41 mitochondrial ribosomal protein L41". Goldschmidt-Reisin S, Kitakawa M, Herfurth E, et al. (1999). " ...
*  Mitochondrial ribosomal protein L42
28S ribosomal protein L42, mitochondrial is a protein that in humans is encoded by the MRPL42 gene. Mammalian mitochondrial ... 2001). "The human mitochondrial ribosomal protein genes: mapping of 54 genes to the chromosomes and implications for human ... "Entrez Gene: MRPL42 mitochondrial ribosomal protein L42". Bonaldo MF, Lennon G, Soares MB (1997). "Normalization and ... 2001). "The large subunit of the mammalian mitochondrial ribosome. Analysis of the complement of ribosomal proteins present". J ...
*  Mitochondrial trifunctional protein deficiency
... ; MTPD". omim.org. Retrieved 2016-11-05. Reference, Genetics Home. "mitochondrial ... The mechanism of this condition indicates that the mitochondrial trifunction protein catalyzes 3 steps in mitochondrial beta- ... The genetics of mitochondrial trifunctional protein deficiency is based on mutations in the HADHA and HADHB genes which cause ... The presentation of mitochondrial trifunctional protein deficiency may begin during infancy, features that occur are: low blood ...
*  Mitochondrial antiviral-signaling protein
... (MAVS) is a protein that in humans is encoded by the MAVS gene. The protein is also ... a mitochondrial antiviral signaling protein that activates NF-kappaB and IRF 3". Cell. 122 (5): 669-82. doi:10.1016/j.cell. ... "Hepatitis C virus protease NS3/4A cleaves mitochondrial antiviral signaling protein off the mitochondria to evade innate ... Xu LG, Wang YY, Han KJ, Li LY, Zhai Z, Shu HB (Sep 2005). "VISA is an adapter protein required for virus-triggered IFN-beta ...
*  Mitochondrial membrane transport protein
Mitochondrial carrier Membrane transport protein Mitochondrial Membrane Transport Proteins at the US National Library of ... Mitochondrial membrane transport proteins are proteins which exist in the membranes of mitochondria and which serve to ... Examples of mitochondrial transport proteins include the following: The mitochondrial permeability transition pore, which opens ... These transport proteins serve to maintain the proper electrical and chemical gradients in mitochondria by keeping ions and ...
*  Mitochondrial ribosomal death-associated protein 3
... the mitochondrial ribosomal death-associated protein 3 family of proteins is a family of conserved proteins which were ... DAP3 has been shown to be a pro-apoptotic factor in the mitochondrial matrix and to be crucial for mitochondrial biogenesis and ... mitochondrial ribosomal protein subunit 29). Kissil JL, Cohen O, Raveh T, Kimchi A (January 1999). "Structure-function analysis ... "The apoptosis mediator mDAP-3 is a novel member of a conserved family of mitochondrial proteins". J. Cell Sci. 113 (20): 3603- ...
*  S-Nitrosoglutathione
Mitochondrial SNO-proteins inhibit Complex I of the electron transport chain; modulate mitochondrial reactive oxygen species ( ... promote selective importation of mitochondrial proteins; and stimulate mitochondrial fission. Altered redox balance plays a ... Piantadosi CA (March 2011). "Regulation of mitochondrial processes by protein S-nitrosylation". Biochim Biophys Acta. 1820 (6 ... Hess DT, Matsumoto A, Kim SO, Marshall HE, Stamler JS (February 2005). "Protein S-nitrosylation: purview and parameters". Nat. ...
*  Beta-ketoacyl-ACP synthase
... and yeast mitochondrial protein CEM1. Beta-ketoacyl synthase contains two protein domains. The active site is located between ... synthase from the mitochondrial type II fatty acid synthase". Protein Science. 16 (2): 261-272. doi:10.1110/ps.062473707. ISSN ... synthase from the mitochondrial type II fatty acid synthase". Protein Science. 16 (2): 261-72. doi:10.1110/ps.062473707. PMC ... "Evolution of acyl-ACP-thioesterases and β-ketoacyl-ACP-synthases revealed by protein-protein interactions". Journal of applied ...
*  Mitochondrial matrix
The mitochondrial DNA only codes for about thirteen proteins that are used in processing mitochondrial transcripts, ribosomal ... and protein subunits found in the protein complexes of the electron transport chain. Matrix (biology) Mitochondrial DNA ... Protein synthesis makes use of mitochondrial DNA, RNA, and tRNA. Regulation of processes makes use of ions(Ca2+/K+/Mg+). ... The protons return to the mitochondrial matrix through the protein ATP synthase.The energy is used in order to rotate ATP ...
*  MFN2
Mitofusin-2 is a mitochondrial membrane protein that participates in mitochondrial fusion and contributes to the maintenance ... Zorzano A, Bach D, Pich S, Palacín M (2004). "[Role of novel mitochondrial proteins in energy balance]". Revista de medicina de ... In addition to the mitofusins, OPA1 regulates inner mitochondrial membrane fusion, and DRP1 is responsible for mitochondrial ... "Mitofusin-2 Determines Mitochondrial Network Architecture and Mitochondrial Metabolism A NOVEL REGULATORY MECHANISM ALTERED IN ...
*  Cytochrome c oxidase
Soltys BJ, Gupta RS (1999). "Mitochondrial proteins at unexpected cellular locations: export of proteins from mitochondria from ... 2012). "MITRAC links mitochondrial protein translocation to respiratory-chain assembly and translational regulation". Cell. 151 ... 2014). "C1orf163/RESA1 is a novel mitochondrial intermembrane space protein connected to respiratory chain assembly". J Mol ... Soltys BJ, Gupta RS (1999). "Mitochondrial proteins at unexpected locations: Are they exported?". Trends Biochem. Sci. 24: 174- ...
*  MTP18
Mitochondrial protein 18 kDa, also known as MTP18, is a human gene. GRCh38: Ensembl release 89: ENSG00000242114 - Ensembl, May ... 2004). "Knockdown of MTP18, a novel phosphatidylinositol 3-kinase-dependent protein, affects mitochondrial morphology and ... MTP18 mitochondrial protein 18 kDa". Bonaldo MF, Lennon G, Soares MB (1997). "Normalization and subtraction: two approaches to ...
*  Ribosome
"Evolution of the mitochondrial protein synthetic machinery". BioSystems. 21 (1): 51-68. doi:10.1016/0303-2647(87)90006-2. PMID ... Each subunit is composed of one or more ribosomal RNA (rRNA) molecules and a variety of ribosomal proteins (r-protein or ... The mitochondrial ribosomes of eukaryotic cells, are produced from mitochondrial genes, and functionally resemble many features ... When a ribosome begins to synthesize proteins that are needed in some organelles, the ribosome making this protein can become " ...
*  Methylglutaconyl-CoA hydratase
AUH is also responsible for regulating mitochondrial protein synthesis and is essential for mitochondrial RNA metabolism, ... "A bifunctional protein regulates mitochondrial protein synthesis". Nucleic Acids Research. 42 (9): 5483-94. doi:10.1093/nar/ ... Kurimoto K, Fukai S, Nureki O, Muto Y, Yokoyama S (Dec 2001). "Crystal structure of human AUH protein, a single-stranded RNA ... These functions allow AUH to show us that there could be a potential connection between mitochondrial metabolism and gene ...
*  Mitointeractome
... is a mitochondrial protein interactome database. Reja, Rohit; et al. (3 December 2009). "MitoInteractome: ... Mitochondrial protein interactome database, and its application in 'aging network' analysis". BMC Genomics. 10. doi:10.1186/ ...
*  Mitochondrial ribosome
Most of mitochondrial proteins are synthesized via cytoplasmic ribosomes. Proteins that are the key components in the electron ... Mitochondria contain around 1000 proteins in yeast and 1500 proteins in human organisms; however only 8 and 13 proteins are ... "Cooperation of protein machineries in mitochondrial protein sorting". Biochimica et Biophysica Acta (BBA) - Molecular Cell ... Mitochondrial ribosome or mitoribosome is a protein complex that is active in mitochondria and functions as a riboprotein for ...
*  PHB2
Coates PJ, Jamieson DJ, Smart K, Prescott AR, Hall PA (Oct 1997). "The prohibitin family of mitochondrial proteins regulate ... 2005). "Towards a proteome-scale map of the human protein-protein interaction network". Nature. 437 (7062): 1173-8. doi:10.1038 ... 2007). "Large-scale mapping of human protein-protein interactions by mass spectrometry". Mol. Syst. Biol. 3 (1): 89. doi: ... "Mammalian prohibitin proteins respond to mitochondrial stress and decrease during cellular senescence". Exp Cell Res. 265 (2): ...
*  Walter L. Miller (endocrinologist)
... and expression of a novel luteinizing hormone-induced mitochondrial protein in MA-10 mouse Leydig tumor cells. Characterization ... "Rapid regulation of steroidogenesis by mitochondrial protein import". Nature. 417 (6884): 87-91. Bibcode:2002Natur.417...87B. ... Bristow, J; Tee, M. K; Gitelman, S. E; Mellon, S. H; Miller, W. L (1993). "Tenascin-X: A novel extracellular matrix protein ... Bose, H. S; Whittal, R. M; Baldwin, M. A; Miller, W. L (1999). "The active form of the steroidogenic acute regulatory protein, ...
*  Electron microscope
ISBN 978-3-319-20800-8. Perkins, G.A. (2014). "The use of miniSOG in the localization of mitochondrial proteins". Methods in ... Williams, R. C.; Wyckoff, R. W. (1945-06-08). "Electron shadow micrography of the tobacco mosaic virus protein". Science. 101 ( ... a preparation method particularly useful for examining lipid membranes and their incorporated proteins in "face on" view. The ... for biological specimens aims to stabilize the specimen's mobile macromolecular structure by chemical crosslinking of proteins ...
*  Proline-rich protein 21
PRR21 may be involved in stress responses that are related to phosphorylation of mitochondrial proteins. The gene is ... Proline-rich protein 21 (PRR21) is a protein of the family of proline-rich proteins. It is encoded by the PRR21 gene, which is ... The protein has a no potential GPI-modification sites. PRR21 is not predicted to interact with any other proteins. PRR21 have ... Kanamaru Y, Sekine S, Ichijo H, Takeda K (2012). "The phosphorylation-dependent regulation of mitochondrial proteins in stress ...
*  MODY 1
... and several proteins involved in glucose and mitochondrial metabolism. Although pancreatic beta cells produce adequate insulin ... This gene codes for HNF4-α protein also known as transcription factor 14 (TCF14). HNF4α controls function of HNF1α (see MODY 3 ...
*  TOMM20
"A human homolog of the mitochondrial protein import receptor Mom19 can assemble with the yeast mitochondrial receptor complex ... "Interactions of the human mitochondrial protein import receptor, hTom20, with precursor proteins in vitro reveal pleiotropic ... Mitochondrial import receptor subunit TOM20 homolog is a protein that in humans is encoded by the TOMM20 gene. GRCh38: Ensembl ... 2000). "Functional analysis of human metaxin in mitochondrial protein import in cultured cells and its relationship with the ...