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MDM10 | Mitochondrial distribution and morphology protein 10; Subunit of both the ERMES and the SAM complex; component of ERMES complex which acts as a molecular tether between the mitochondria and the ER, necessary for efficient phospholipid exchange between organelles and for mitophagy; SAM/TOB complex component that functions in the assembly of outer membrane beta-barrel proteins; involved in mitochondrial inheritance and morphology; ERMES complex is often co-localized with peroxisomes and concentrated areas of pyruvate dehydrogenase; Belongs to the MDM10 family. (493 aa) | ||||
CDS1 | Phosphatidate cytidylyltransferase (CDP-diglyceride synthetase); an enzyme that catalyzes that conversion of CTP + phosphate into diphosphate + CDP-diaclglyerol, a critical step in the synthesis of all major yeast phospholipids; human homolog CDS1 can complement yeast cds1 null mutant. (457 aa) | ||||
PGS1 | CDP-diacylglycerol--glycerol-3-phosphate 3-phosphatidyltransferase; Phosphatidylglycerolphosphate synthase; catalyzes the synthesis of phosphatidylglycerolphosphate from CDP-diacylglycerol and sn-glycerol 3-phosphate in the first committed and rate-limiting step of cardiolipin biosynthesis. (521 aa) | ||||
CRD1 | Cardiolipin synthase; produces cardiolipin, which is a phospholipid of the mitochondrial inner membrane that is required for normal mitochondrial membrane potential and function and for correct integration of membrane-multispanning proteins into the mitochondrial outer membrane; required to maintain tubular mitochondrial morphology and functions in mitochondrial fusion; also required for normal vacuolar ion homeostasis; Belongs to the CDP-alcohol phosphatidyltransferase class-I family. (283 aa) | ||||
TPI1 | Triose phosphate isomerase, abundant glycolytic enzyme; mRNA half-life is regulated by iron availability; transcription is controlled by activators Reb1p, Gcr1p, and Rap1p through binding sites in the 5' non-coding region; inhibition of Tpi1p activity by PEP (phosphoenolpyruvate) stimulates redox metabolism in respiring cells; E104D mutation in human homolog TPI1 causes a rare autosomal disease; human TPI1 can complement yeast null mutant. (248 aa) | ||||
UPS3 | Mitochondrial protein of unknown function; similar to Ups1p and Ups2p which are involved in regulation of mitochondrial cardiolipin and phosphatidylethanolamine levels; null is viable but interacts synthetically with ups1 and ups2 mutations; UPS3 has a paralog, UPS2, that arose from the whole genome duplication. (179 aa) | ||||
CYC7 | Cytochrome c isoform 2, expressed under hypoxic conditions; also known as iso-2-cytochrome c; electron carrier of the mitochondrial intermembrane space that transfers electrons from ubiquinone-cytochrome c oxidoreductase to cytochrome c oxidase during cellular respiration; protein abundance increases in response to DNA replication stress; CYC7 has a paralog, CYC1, that arose from the whole genome duplication. (113 aa) | ||||
MDM34 | Mitochondrial distribution and morphology protein 34; Mitochondrial component of the ERMES complex; links the ER to mitochondria and may promote inter-organellar calcium and phospholipid exchange as well as coordinating mitochondrial DNA replication and growth; required for mitophagy; ERMES complex is often co-localized with peroxisomes and with concentrated areas of pyruvate dehydrogenase. (459 aa) | ||||
TAM41 | Mitochondrial phosphatidate cytidylyltransferase (CDP-DAG synthase); required for cardiolipin biosynthesis; viability of null mutant is strain-dependent; mRNA is targeted to the bud; mutant displays defect in mitochondrial protein import, likely due to altered membrane lipid composition; Belongs to the TAM41 family. (385 aa) | ||||
TOM20 | Mitochondrial import receptor subunit TOM20; Component of the TOM (translocase of outer membrane) complex; responsible for recognition and initial import steps for all mitochondrially directed proteins; acts as a receptor for incoming precursor proteins. (183 aa) | ||||
CLD1 | Mitochondrial cardiolipin-specific phospholipase; functions upstream of Taz1p to generate monolyso-cardiolipin; transcription increases upon genotoxic stress; involved in restricting Ty1 transposition; has homology to mammalian CGI-58; Belongs to the peptidase S33 family. ABHD4/ABHD5 subfamily. (445 aa) | ||||
CHO2 | Phosphatidylethanolamine methyltransferase (PEMT); catalyzes the first step in the conversion of phosphatidylethanolamine to phosphatidylcholine during the methylation pathway of phosphatidylcholine biosynthesis. (869 aa) | ||||
PSD2 | Phosphatidylserine decarboxylase 2 alpha chain; Phosphatidylserine decarboxylase of the Golgi and vacuolar membranes; converts phosphatidylserine to phosphatidylethanolamine; controls vacuolar membrane phospholipid content by regulating phospholipids in compartments that will eventually give rise to the vacuole; loss of Psd2p causes a specific reduction in vacuolar membrane PE levels while total PE levels are not significantly affected. (1138 aa) | ||||
GEP4 | Mitochondrial phosphatidylglycerophosphatase (PGP phosphatase); dephosphorylates phosphatidylglycerolphosphate to generate phosphatidylglycerol, an essential step during cardiolipin biosynthesis; null mutant is sensitive to tunicamycin, DTT; Belongs to the GEP4 family. (185 aa) | ||||
MET30 | F-box protein containing five copies of the WD40 motif; controls cell cycle function, sulfur metabolism, and methionine biosynthesis as part of the ubiquitin ligase complex; interacts with and regulates Met4p, localizes within the nucleus; dissociation of Met30p from SCF complex in response to cadmium stress is regulated by Cdc48p. (640 aa) | ||||
CYC1 | Cytochrome c, isoform 1; also known as iso-1-cytochrome c; electron carrier of mitochondrial intermembrane space that transfers electrons from ubiquinone-cytochrome c oxidoreductase to cytochrome c oxidase during cellular respiration; CYC1 has a paralog, CYC7, that arose from the whole genome duplication; human homolog CYC1 can complement yeast null mutant; mutations in human CYC1 cause insulin-responsive hyperglycemia. (109 aa) | ||||
OPI3 | Phosphatidyl-N-methylethanolamine N-methyltransferase; Methylene-fatty-acyl-phospholipid synthase; catalyzes the last two steps in phosphatidylcholine biosynthesis; also known as phospholipid methyltransferase. (206 aa) | ||||
MDM35 | Mitochondrial distribution and morphology protein 35; Mitochondrial intermembrane space protein; forms complex with Ups2p that transfers phosphatidylserine from outer membrane to inner membrane for phosphatidylethanolamine synthesis; mutation affects mitochondrial distribution and morphology; contains twin cysteine-x9-cysteine motifs; protein abundance increases in response to DNA replication stress. (86 aa) | ||||
SPO14 | Phospholipase D; catalyzes the hydrolysis of phosphatidylcholine, producing choline and phosphatidic acid; involved in Sec14p-independent secretion; required for meiosis and spore formation; differently regulated in secretion and meiosis; participates in transcription initiation and/or early elongation of specific genes; interacts with "foot domain" of RNA polymerase II; deletion results in abnormal CTD-Ser5 phosphorylation of RNA polymerase II at specific promoter regions. (1683 aa) | ||||
UPS2 | Mitochondrial intermembrane space protein; involved in phospholipid metabolism; forms complex with Mdm35p that transfers phosphatidylserine from outer membrane to inner membrane for phosphatidylethanolamine synthesis; null mutant has defects in mitochondrial morphology; similar to Ups1p, Ups3p and to human PRELI; UPS2 has a paralog, UPS3, that arose from the whole genome duplication; Belongs to the slowmo family. (230 aa) | ||||
UPS1 | Protein UPS1, mitochondrial; Phosphatidic acid transfer protein; plays a role in phospholipid metabolism by transporting phosphatidic acid from the outer to the inner mitochondrial membrane; localizes to the mitochondrial intermembrane space; null mutant has altered cardiolipin and phosphatidic acid levels; ortholog of human PRELI; Belongs to the slowmo family. (175 aa) | ||||
PAH1 | Phosphatidic acid phosphohydrolase 1; Mg2+-dependent phosphatidate (PA) phosphatase; dephosphorylates PA to yield diacylglycerol; regulates phospholipid synthesis, nuclear/ER membrane growth, lipid droplet formation, triacylglycerol synthesis, vacuolar homeostasis and cell wall integrity; phosphorylated by Pho85p/Pho80p, Cdc28p/Cyclin B, PKA, PKC, and CKII, regulating activity, localization, and proteosomal degradation; homolog of mammalian lipins 1 and 2; human homologs LPIN1, LPIN2, LPIN3 complement the null. (862 aa) | ||||
MET4 | Leucine-zipper transcriptional activator; responsible for regulation of sulfur amino acid pathway; requires different combinations of auxiliary factors Cbf1p, Met28p, Met31p and Met32p; feedforward loop exists in the regulation of genes controlled by Met4p and Met32p; can be ubiquitinated by ubiquitin ligase SCF-Met30p, is either degraded or maintained in an inactive state; regulates degradation of its own DNA-binding cofactors by targeting them to SCF-Met30p; Belongs to the bZIP family. (672 aa) | ||||
PSD1 | Phosphatidylserine decarboxylase of the mitochondrial inner membrane; converts phosphatidylserine to phosphatidylethanolamine; regulates mitochondrial fusion and morphology by affecting lipid mixing in the mitochondrial membrane and by influencing the ratio of long to short forms of Mgm1p; partly exposed to the mitochondrial intermembrane space; autocatalytically processed; Belongs to the phosphatidylserine decarboxylase family. PSD-B subfamily. Eukaryotic type I sub-subfamily. (500 aa) | ||||
ATP23 | Putative metalloprotease of the mitochondrial inner membrane; required for processing of Atp6p; has an additional role in assembly of the F0 sector of the F1F0 ATP synthase complex; substrate of the Mia40p-Erv1p disulfide relay system, and folding is assisted by Mia40p; Belongs to the peptidase M76 family. (270 aa) | ||||
MDM12 | Mitochondrial distribution and morphology protein 12; Mitochondrial outer membrane protein, ERMES complex subunit; required for transmission of mitochondria to daughter cells; required for mitophagy; may influence import and assembly of outer membrane beta-barrel proteins; ERMES complex is often co-localized with peroxisomes and with concentrated areas of pyruvate dehydrogenase; Belongs to the MDM12 family. (271 aa) | ||||
ALE1 | Broad-specificity lysophospholipid acyltransferase; part of MBOAT family of membrane-bound O-acyltransferases; key component of Lands cycle; may have role in fatty acid exchange at sn-2 position of mature glycerophospholipids; Belongs to the membrane-bound acyltransferase family. (619 aa) | ||||
MGM1 | Mitochondrial GTPase, present in complex with Ugo1p and Fzo1p; required for mitochondrial morphology, fusion, and genome maintenance; promotes membrane bending; exists as long and short form with different distributions; ratio of long to short forms is regulated by Psd1p; homolog of human OPA1 involved in autosomal dominant optic atrophy. (881 aa) | ||||
YME1 | Catalytic subunit of i-AAA protease complex; complex is located in mitochondrial inner membrane; responsible for degradation of unfolded or misfolded mitochondrial gene products; serves as nonconventional translocation motor to pull PNPase into intermembrane space; also has role in intermembrane space protein folding; mutation causes elevated rate of mitochondrial turnover; human homolog YME1L1 can complement yeast null mutant; In the C-terminal section; belongs to the peptidase M41 family. (747 aa) | ||||
TAZ1 | Lyso-phosphatidylcholine acyltransferase; required for normal phospholipid content of mitochondrial membranes; major determinant of the final acyl chain composition of the mitochondrial-specific phospholipid cardiolipin; mutations in human ortholog tafazzin (TAZ) cause Barth syndrome, a rare X-linked disease characterized by skeletal and cardiomyopathy and bouts of cyclic neutropenia; a specific splice variant of human TAZ can complement yeast null mutant. (381 aa) |