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| MRPS10 | Mitochondrial ribosomal protein S10; Belongs to the universal ribosomal protein uS10 family (201 aa) | |||
| SLC25A1 | Tricarboxylate transport protein, mitochondrial; Involved in citrate-H(+)/malate exchange. 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; Solute carriers (311 aa) | |||
| NIPSNAP1 | Nipsnap homolog 1 (284 aa) | |||
| ECH1 | Delta(3,5)-Delta(2,4)-dienoyl-CoA isomerase, mitochondrial; Isomerization of 3-trans,5-cis-dienoyl-CoA to 2-trans,4- trans-dienoyl-CoA (328 aa) | |||
| C1QBP | Complement component 1 Q subcomponent-binding protein, mitochondrial; Is believed to be a multifunctional and multicompartmental protein involved in inflammation and infection processes, ribosome biogenesis, regulation of apoptosis, transcriptional regulation and pre-mRNA splicing. At the cell surface is thought to act as an endothelial receptor for plasma proteins of the complement and kallikrein-kinin cascades. Putative receptor for C1q; specifically binds to the globular "heads" of C1q thus inhibiting C1; may perform the receptor function through a complex with C1qR/CD93. In complex [...] (282 aa) | |||
| COX6A1 | Cytochrome c oxidase subunit 6A1, mitochondrial; This protein is one of the nuclear-coded polypeptide chains of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport (109 aa) | |||
| THG1L | Probable tRNA(His) guanylyltransferase; Adds a GMP to the 5’-end of tRNA(His) after transcription and RNase P cleavage. This step is essential for proper recognition of the tRNA and for the fidelity of protein synthesis (298 aa) | |||
| PMPCB | Mitochondrial-processing peptidase subunit beta; Cleaves presequences (transit peptides) from mitochondrial protein precursors; M16 metallopeptidases (489 aa) | |||
| NDUFA2 | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2; Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (99 aa) | |||
| GRSF1 | G-rich sequence factor 1; Regulator of post-transcriptional mitochondrial gene expression, required for assembly of the mitochondrial ribosome and for recruitment of mRNA and lncRNA. Binds RNAs containing the 14 base G-rich element. Preferentially binds RNAs transcribed from three contiguous genes on the light strand of mtDNA, the ND6 mRNA, and the long non-coding RNAs for MT-CYB and MT-ND5, each of which contains multiple consensus binding sequences (480 aa) | |||
| ERAL1 | GTPase Era, mitochondrial; Probable GTPase that plays a role in the mitochondrial ribosomal small subunit assembly. Specifically binds the 12S mitochondrial rRNA (12S mt-rRNA) to a 33 nucleotide section delineating the 3’ terminal stem-loop region. May act as a chaperone that protects the 12S mt-rRNA on the 28S mitoribosomal subunit during ribosomal small subunit assembly (437 aa) | |||
| SCO1 | Protein SCO1 homolog, mitochondrial; Thought to play a role in cellular copper homeostasis, mitochondrial redox signaling or insertion of copper into the active site of COX; Belongs to the SCO1/2 family (301 aa) | |||
| TBRG4 | Protein TBRG4; May play a role in cell cycle progression; FASTK mitochondrial RNA binding family (631 aa) | |||
| NDUFS3 | NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial; Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity); NADH-ubiquinone oxidoreductase core subunits (264 aa) | |||
| IDE | Insulin-degrading enzyme; Plays a role in the cellular breakdown of insulin, IAPP, glucagon, bradykinin, kallidin and other peptides, and thereby plays a role in intercellular peptide signaling. Degrades amyloid formed by APP and IAPP. May play a role in the degradation and clearance of naturally secreted amyloid beta-protein by neurons and microglia; M16 metallopeptidases (1019 aa) | |||
| UQCRC2 | Cytochrome b-c1 complex subunit 2, mitochondrial; This is a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain. The core protein 2 is required for the assembly of the complex; M16 metallopeptidases (453 aa) | |||
| AFG3L2 | AFG3-like protein 2; ATP-dependent protease which is essential for axonal and neuron development. In neurons, mediates degradation of SMDT1/EMRE before its assembly with the uniporter complex, limiting the availability of SMDT1/EMRE for MCU assembly and promoting efficient assembly of gatekeeper subunits with MCU. Required for the maturation of paraplegin (SPG7) after its cleavage by mitochondrial-processing peptidase (MPP), converting it into a proteolytically active mature form (By similarity); In the N-terminal section; belongs to the AAA ATPase family (797 aa) | |||
| BCKDHA | 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components- branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3) (445 aa) | |||
| TIMM44 | Mitochondrial import inner membrane translocase subunit TIM44; Essential component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner. Recruits mitochondrial HSP70 to drive protein translocation into the matrix using ATP as an energy source; Belongs to the Tim44 family (452 aa) | |||
| NDUFC2 | NADH dehydrogenase [ubiquinone] 1 subunit C2; Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (119 aa) | |||
| NARS2 | Probable asparagine--tRNA ligase, mitochondrial; asparaginyl-tRNA synthetase 2, mitochondrial; Belongs to the class-II aminoacyl-tRNA synthetase family (477 aa) | |||
| MTERF3 | Transcription termination factor 3, mitochondrial; Binds promoter DNA and regulates initiation of transcription. Required for normal mitochondrial transcription and translation, and for normal assembly of mitochondrial respiratory complexes. Required for normal mitochondrial function (By similarity). Maintains 16S rRNA levels and functions in mitochondrial ribosome assembly by regulating the biogenesis of the 39S ribosomal subunit (By similarity); Belongs to the mTERF family (417 aa) | |||
| COX6A2 | Cytochrome c oxidase subunit 6A2, mitochondrial; This protein is one of the nuclear-coded polypeptide chains of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport (97 aa) | |||
| ATP5O | ATP synthase subunit O, mitochondrial; Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase or Complex V) produces ATP from ADP in the presence of a proton gradient across the membrane which is generated by electron transport complexes of the respiratory chain. F-type ATPases consist of two structural domains, F(1) - containing the extramembraneous catalytic core and F(0) - containing the membrane proton channel, linked together by a central stalk and a peripheral stalk. During catalysis, ATP synthesis in the catalytic domain of F(1) is coupled via a rotary mechanism of the centr [...] (213 aa) | |||
| SMOC1 | SPARC-related modular calcium-binding protein 1; Plays essential roles in both eye and limb development. Probable regulator of osteoblast differentiation; SPARC family (435 aa) | |||
| CISD3 | CDGSH iron-sulfur domain-containing protein 3, mitochondrial; CDGSH iron sulfur domain containing; Belongs to the CISD protein family (127 aa) | |||
