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CLPX | Caseinolytic mitochondrial matrix peptidase chaperone subunit. (690 aa) | ||||
TAF10 | Transcription initiation factor TFIID subunit 10; TFIID is a multimeric protein complex that plays a central role in mediating promoter responses to various activators and repressors; Belongs to the TAF10 family. (218 aa) | ||||
ASH2L | ASH2 like, histone lysine methyltransferase complex subunit. (629 aa) | ||||
LSM1 | U6 snRNA-associated Sm-like protein LSm1; Plays a role in the degradation of histone mRNAs, the only eukaryotic mRNAs that are not polyadenylated (By similarity). Probably also part of an LSm subunits-containing complex involved in the general process of mRNA degradation (By similarity). (133 aa) | ||||
EIF1 | Eukaryotic translation initiation factor 1; Necessary for scanning and involved in initiation site selection. Promotes the assembly of 48S ribosomal complexes at the authentic initiation codon of a conventional capped mRNA (By similarity); Belongs to the SUI1 family. (113 aa) | ||||
PFKP | ATP-dependent 6-phosphofructokinase; Catalyzes the phosphorylation of D-fructose 6-phosphate to fructose 1,6-bisphosphate by ATP, the first committing step of glycolysis; Belongs to the phosphofructokinase type A (PFKA) family. ATP-dependent PFK group I subfamily. Eukaryotic two domain clade 'E' sub-subfamily. (960 aa) | ||||
KCTD10 | Potassium channel tetramerization domain containing 10. (312 aa) | ||||
GPT2 | Glutamic--pyruvic transaminase 2. (567 aa) | ||||
NME6 | Nucleoside diphosphate kinase. (186 aa) | ||||
YBEY | YbeY metalloendoribonuclease. (385 aa) | ||||
KEAP1 | Kelch like ECH associated protein 1. (624 aa) | ||||
MTHFD2 | Bifunctional methylenetetrahydrofolate dehydrogenase/cyclohydrolase, mitochondrial; Although its dehydrogenase activity is NAD-specific, it can also utilize NADP at a reduced efficiency. (378 aa) | ||||
TYMS | Thymidylate synthase; Contributes to the de novo mitochondrial thymidylate biosynthesis pathway. (354 aa) | ||||
GPT | Alanine aminotransferase 1; Catalyzes the reversible transamination between alanine and 2-oxoglutarate to form pyruvate and glutamate. Participates in cellular nitrogen metabolism and also in liver gluconeogenesis starting with precursors transported from skeletal muscles (By similarity). Belongs to the class-I pyridoxal-phosphate-dependent aminotransferase family. Alanine aminotransferase subfamily. (820 aa) | ||||
GLS | Glutaminase. (603 aa) | ||||
SLC20A1 | Phosphate transporter; Sodium-phosphate symporter which plays a fundamental housekeeping role in phosphate transport. Belongs to the inorganic phosphate transporter (PiT) (TC 2.A.20) family. (681 aa) | ||||
FLCN | Folliculin; May be a tumor suppressor. May be involved in energy and/or nutrient sensing through the AMPK and mTOR signaling pathways. May regulate phosphorylation of RPS6KB1. (579 aa) | ||||
MTRR | Methionine synthase reductase; Involved in the reductive regeneration of cob(I)alamin (vitamin B12) cofactor required for the maintenance of methionine synthase in a functional state. Necessary for utilization of methylgroups from the folate cycle, thereby affecting transgenerational epigenetic inheritance. Folate pathway donates methyl groups necessary for cellular methylation and affects different pathways such as DNA methylation, possibly explaining the transgenerational epigenetic inheritance effects. (695 aa) | ||||
EMC2 | ER membrane protein complex subunit 2; Belongs to the EMC2 family. (297 aa) | ||||
NAIF1 | Nuclear apoptosis-inducing factor 1; Induces apoptosis. (327 aa) | ||||
AARS2 | Alanyl-tRNA synthetase 2, mitochondrial; Catalyzes the attachment of alanine to tRNA(Ala) in a two- step reaction: alanine is first activated by ATP to form Ala-AMP and then transferred to the acceptor end of tRNA(Ala). Also edits incorrectly charged tRNA(Ala) via its editing domain. Belongs to the class-II aminoacyl-tRNA synthetase family. (983 aa) | ||||
SLC7A1 | Solute carrier family 7 member 1. (629 aa) | ||||
PARL | Presenilins-associated rhomboid-like protein, mitochondrial; Required for the control of apoptosis during postnatal growth. Essential for proteolytic processing of an antiapoptotic form of OPA1 which prevents the release of mitochondrial cytochrome c in response to intrinsic apoptotic signals (By similarity). Required for the maturation of PINK1 into its 52kDa mature form after its cleavage by mitochondrial-processing peptidase (MPP). Promotes changes in mitochondria morphology regulated by phosphorylation of P-beta domain (By similarity). (403 aa) | ||||
CLPP | ATP-dependent Clp protease proteolytic subunit, mitochondrial; Protease component of the Clp complex that cleaves peptides and various proteins in an ATP-dependent process. Has low peptidase activity in the absence of CLPX. The Clp complex can degrade CSN1S1, CSN2 and CSN3, as well as synthetic peptides (in vitro) and may be responsible for a fairly general and central housekeeping function rather than for the degradation of specific substrates. Cleaves PINK1 in the mitochondrion. (272 aa) | ||||
MTOR | Serine/threonine-protein kinase mTOR; Belongs to the PI3/PI4-kinase family. (2550 aa) | ||||
RBBP5 | RB binding protein 5, histone lysine methyltransferase complex subunit. (538 aa) | ||||
MMACHC | Methylmalonic aciduria and homocystinuria type C protein homolog; Catalyzes the reductive dealkylation of cyanocobalamin to cob(II)alamin, using FAD or FMN as cofactor and NADPH as cosubstrate. Can also catalyze the glutathione-dependent reductive demethylation of methylcobalamin, and, with much lower efficiency, the glutathione- dependent reductive demethylation of adenosylcobalamin. Under anaerobic conditions cob(I)alamin is the first product; it is highly reactive and is converted to aquocob(II)alamin in the presence of oxygen. Binds cyanocobalamin, adenosylcobalamin, methylcobalami [...] (280 aa) | ||||
ALB | Serum albumin; Serum albumin, the main protein of plasma, has a good binding capacity for water, Ca(2+), Na(+), K(+), fatty acids, hormones, bilirubin and drugs. Its main function is the regulation of the colloidal osmotic pressure of blood. Major zinc transporter in plasma, typically binds about 80% of all plasma zinc (By similarity). Major calcium and magnesium transporter in plasma, binds approximately 45% of circulating calcium and magnesium in plasma (Probable). Potentially has more than two calcium-binding sites and might additionally bind calcium in a non-specific manner. The sh [...] (607 aa) | ||||
SLC25A37 | Mitoferrin-1; Mitochondrial iron transporter that specifically mediates iron uptake in developing erythroid cells, thereby playing an essential role in heme biosynthesis. The iron delivered into the mitochondria, presumably as Fe(2+), is then probably delivered to ferrochelatase to catalyze Fe(2+) incorporation into protoprophyrin IX to make heme (By similarity); Belongs to the mitochondrial carrier (TC 2.A.29) family. (338 aa) | ||||
EIF1B | Eukaryotic translation initiation factor 1B. (113 aa) | ||||
AARS1 | Alanyl-tRNA synthetase; Catalyzes the attachment of alanine to tRNA(Ala) in a two- step reaction: alanine is first activated by ATP to form Ala-AMP and then transferred to the acceptor end of tRNA(Ala). Also edits incorrectly charged tRNA(Ala) via its editing domain. Belongs to the class-II aminoacyl-tRNA synthetase family. (968 aa) | ||||
LETM1 | Mitochondrial proton/calcium exchanger protein; Mitochondrial proton/calcium antiporter that mediates proton- dependent calcium efflux from mitochondrion (By similarity). Crucial for the maintenance of mitochondrial tubular networks and for the assembly of the supercomplexes of the respiratory chain (By similarity). Required for the maintenance of the tubular shape and cristae organization (By similarity). In contrast to SLC8B1/NCLX, does not constitute the major factor for mitochondrial calcium extrusion (By similarity); Belongs to the LETM1 family. (786 aa) | ||||
MPC2 | Mitochondrial pyruvate carrier; Mediates the uptake of pyruvate into mitochondria. Belongs to the mitochondrial pyruvate carrier (MPC) (TC 2.A.105) family. (127 aa) | ||||
TBC1D31 | TBC1 domain family member 31. (1063 aa) | ||||
SLC25A11 | Mitochondrial 2-oxoglutarate/malate carrier protein; Catalyzes the transport of 2-oxoglutarate across the inner mitochondrial membrane in an electroneutral exchange for malate or other dicarboxylic acids, and plays an important role in several metabolic processes, including the malate-aspartate shuttle, the oxoglutarate/isocitrate shuttle, in gluconeogenesis from lactate, and in nitrogen metabolism. Maintains mitochondrial fusion and fission events, and the organization and morphology of cristae. Involved in the regulation of apoptosis; Belongs to the mitochondrial carrier (TC 2.A.29) [...] (314 aa) | ||||
SHMT2 | Serine hydroxymethyltransferase, mitochondrial; Catalyzes the cleavage of serine to glycine accompanied with the production of 5,10-methylenetetrahydrofolate, an essential intermediate for purine biosynthesis. Serine provides the major source of folate one-carbon in cells by catalyzing the transfer of one carbon from serine to tetrahydrofolate. Contributes to the de novo mitochondrial thymidylate biosynthesis pathway via its role in glycine and tetrahydrofolate metabolism: thymidylate biosynthesis is required to prevent uracil accumulation in mtDNA. Also required for mitochondrial tran [...] (504 aa) | ||||
MPC1 | Mitochondrial pyruvate carrier 1; Mediates the uptake of pyruvate into mitochondria. Belongs to the mitochondrial pyruvate carrier (MPC) (TC 2.A.105) family. (109 aa) | ||||
SLC25A46 | Solute carrier family 25 member 46; Belongs to the mitochondrial carrier (TC 2.A.29) family. (405 aa) | ||||
GHITM | Growth hormone inducible transmembrane protein; Belongs to the BI1 family. (345 aa) | ||||
SLC25A32 | Solute carrier family 25 member 32; Belongs to the mitochondrial carrier (TC 2.A.29) family. (317 aa) | ||||
MTHFD1L | Monofunctional C1-tetrahydrofolate synthase, mitochondrial; May provide the missing metabolic reaction required to link the mitochondria and the cytoplasm in the mammalian model of one-carbon folate metabolism in embryonic an transformed cells complementing thus the enzymatic activities of MTHFD2; In the N-terminal section; belongs to the tetrahydrofolate dehydrogenase/cyclohydrolase family. (975 aa) | ||||
UBC | Ubiquitin-related; [Ubiquitin]: Exists either covalently attached to another protein, or free (unanchored). When covalently bound, it is conjugated to target proteins via an isopeptide bond either as a monomer (monoubiquitin), a polymer linked via different Lys residues of the ubiquitin (polyubiquitin chains) or a linear polymer linked via the initiator Met of the ubiquitin (linear polyubiquitin chains). Polyubiquitin chains, when attached to a target protein, have different functions depending on the Lys residue of the ubiquitin that is linked: Lys-6-linked may be involved in DNA repa [...] (690 aa) | ||||
TENT4B | Terminal nucleotidyltransferase 4B. (666 aa) | ||||
PDHB | Pyruvate dehydrogenase E1 component subunit beta, mitochondrial; The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle. (384 aa) | ||||
SLC25A1 | Tricarboxylate transport protein, mitochondrial; Citrate transporter that mediates the exchange of mitochondrial citrate for cytosolic malate. Also able to mediate the exchange of citrate for isocitrate, phosphoenolpyruvate, cis- but not trans-aconitate and to a lesser extend maleate and succinate. Important for the bioenergetics of hepatic cells as it provides a carbon source for fatty acid and sterol biosyntheses, and NAD(+) for the glycolytic pathway. Required for proper neuromuscular junction formation. Belongs to the mitochondrial carrier (TC 2.A.29) family. (312 aa) | ||||
UFM1 | Ubiquitin-fold modifier 1; Ubiquitin-like modifier which can be covalently attached via an isopeptide bond to substrate proteins as a monomer or a lysine- linked polymer. The so-called ufmylation, requires the UFM1-activating E1 enzyme UBA5, the UFM1-conjugating E2 enzyme UFC1, and the UFM1- ligase E3 enzyme UFL1. This post-translational modification on lysine residues of proteins may play a crucial role in a number of cellular processes. TRIP4 ufmylation may for instance play a role in nuclear receptors-mediated transcription. Other substrates may include DDRGK1 with which it may play [...] (85 aa) | ||||
ACLY | ATP-citrate synthase; Catalyzes the cleavage of citrate into oxaloacetate and acetyl-CoA, the latter serving as common substrate for de novo cholesterol and fatty acid synthesis; In the N-terminal section; belongs to the succinate/malate CoA ligase beta subunit family. (1101 aa) | ||||
NADK2 | NAD kinase 2, mitochondrial; Mitochondrial NAD(+) kinase that phosphorylates NAD(+) to yield NADP(+). Can use both ATP or inorganic polyphosphate as the phosphoryl donor. (464 aa) | ||||
GAPDH | Glyceraldehyde-3-phosphate dehydrogenase; Has both glyceraldehyde-3-phosphate dehydrogenase and nitrosylase activities, thereby playing a role in glycolysis and nuclear functions, respectively. Glyceraldehyde-3-phosphate dehydrogenase is a key enzyme in glycolysis that catalyzes the first step of the pathway by converting D-glyceraldehyde 3-phosphate (G3P) into 3-phospho-D-glyceroyl phosphate. Modulates the organization and assembly of the cytoskeleton. Facilitates the CHP1-dependent microtubule and membrane associations through its ability to stimulate the binding of CHP1 to microtubu [...] (376 aa) | ||||
EMC1 | ER membrane protein complex subunit 1. (998 aa) | ||||
FBXW11 | F-box and WD repeat domain containing 11. (563 aa) | ||||
MTR | Methionine synthase; Catalyzes the transfer of a methyl group from methyl- cobalamin to homocysteine, yielding enzyme-bound cob(I)alamin and methionine. Subsequently, remethylates the cofactor using methyltetrahydrofolate (By similarity). (1265 aa) | ||||
SFXN1 | Sideroflexin-1; Mitochondrial serine transporter that mediates transport of serine into mitochondria, an important step of the one-carbon metabolism pathway. Mitochondrial serine is converted to glycine and formate, which then exits to the cytosol where it is used to generate the charged folates that serve as one-carbon donors. Transports both D- serine and L-serine. Also able to transport other amino-acids, such as alanine (By similarity). May be indirectly involved in the transport of a component required for iron utilization into or out of the mitochondria (By similarity); Belongs t [...] (338 aa) | ||||
GLS-2 | ANK_REP_REGION domain-containing protein. (93 aa) | ||||
RAP2A | RAP2A, member of RAS oncogene family. (231 aa) | ||||
BCL2L1 | BCL2-like 1 transcript variant 1. (233 aa) | ||||
MCUR1 | Mitochondrial calcium uniporter regulator 1. (367 aa) | ||||
CCDC58 | Coiled-coil domain-containing protein 58; Belongs to the CCDC58 family. (144 aa) | ||||
FNIP1 | Folliculin interacting protein 1. (1169 aa) | ||||
METAP1 | Methionine aminopeptidase 1; Cotranslationally removes the N-terminal methionine from nascent proteins. The N-terminal methionine is often cleaved when the second residue in the primary sequence is small and uncharged (Met- Ala-, Cys, Gly, Pro, Ser, Thr, or Val). (389 aa) | ||||
NADK | NAD kinase. (522 aa) |