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| | kat-1 | Acetyl-CoA acetyltransferase homolog, mitochondrial; Plays a role in ketone body metabolism (By similarity). Has an effect on lysosome related organelles (LRO) function, in a pathway with serotonin. Acts downstream of sir-2.1 to regulate aging and stress resistance. Belongs to the thiolase-like superfamily. Thiolase family. (407 aa) |
| | mrps-31 | 28S ribosomal protein S31, mitochondrial; Belongs to the mitochondrion-specific ribosomal protein mS31 family. (383 aa) |
| | lonp-1 | Lon protease homolog, mitochondrial; ATP-dependent serine protease that mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides as well as certain short-lived regulatory proteins in the mitochondrial matrix. May also have a chaperone function in the assembly of inner membrane protein complexes. Participates in the regulation of mitochondrial gene expression and in the maintenance of the integrity of the mitochondrial genome. Binds to mitochondrial DNA in a site-specific manner. Involved in the degradation of transcription factor atfs-1 in the mi [...] (971 aa) |
| | sdha-2 | Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial; Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). (640 aa) |
| | C34C12.8 | GrpE protein homolog, mitochondrial; 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. Seems to control the nucleotide-dependent binding of mitochondrial HSP70 to substrate proteins (By similarity). (237 aa) |
| | prx-10 | Tryptophan--tRNA ligase, mitochondrial; Catalyzes the attachment of tryptophan to tRNA(Trp). Belongs to the class-I aminoacyl-tRNA synthetase family. (360 aa) |
| | atp-2 | ATP synthase subunit beta, 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 c [...] (538 aa) |
| | coq-8 | Atypical kinase coq-8, mitochondrial; Atypical kinase involved in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration. Its substrate specificity is unclear: either acts as protein kinase that phosphorylates other proteins in the CoQ complex to stabilize their interactions or acts as a small molecule kinase that phosphorylates a prenyl lipid in the ubiquinone biosynthesis pathway. (755 aa) |
| | idhb-1 | Probable isocitrate dehydrogenase [NAD] subunit beta, mitochondrial; Belongs to the isocitrate and isopropylmalate dehydrogenases family. (379 aa) |
| | sod-2 | Superoxide dismutase [Mn] 1, mitochondrial; Destroys superoxide anion radicals which are normally produced within the cells and which are toxic to biological systems. (221 aa) |
| | mrps-9 | Probable 40S ribosomal protein S9, mitochondrial; Belongs to the universal ribosomal protein uS9 family. (392 aa) |
| | hach-1 | 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite; Belongs to the enoyl-CoA hydratase/isomerase family. (386 aa) |
| | acl-6 | Probable glycerol-3-phosphate acyltransferase, mitochondrial; Belongs to the GPAT/DAPAT family. (718 aa) |
| | hsp-6 | Heat shock 70 kDa protein F, mitochondrial; Belongs to the heat shock protein 70 family. (657 aa) |
| | cps-6 | Endonuclease G, mitochondrial; Endonuclease important for programmed cell death; it mediates apoptotic DNA fragmentation. (308 aa) |
| | gsr-1 | Glutathione reductase, mitochondrial; Maintains high levels of reduced glutathione in the cytosol. Involved in resistance to oxidative stress and starvation. Together with thioredoxin reductase txtr- 1, required for the reduction of disulfide groups in the cuticle during molting. (473 aa) |
| | nuaf-1 | Probable complex I intermediate-associated protein 30, mitochondrial; Chaperone protein involved in the assembly of the mitochondrial NADH:ubiquinone oxidoreductase complex (complex I). Required for normal growth and reproduction. (340 aa) |
| | sucg-1 | Succinate--CoA ligase [GDP-forming] subunit beta, mitochondrial; GTP-specific succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of GTP and thus represents the only step of substrate-level phosphorylation in the TCA. The beta subunit provides nucleotide specificity of the enzyme and binds the substrate succinate, while the binding sites for coenzyme A and phosphate are found in the alpha subunit. (415 aa) |
| | gcsh-1 | Glycine cleavage system H protein; The H protein shuttles the methylamine group of glycine from the P protein to the T protein; Belongs to the GcvH family. (148 aa) |
| | pink-1 | Serine/threonine-protein kinase pink-1, mitochondrial; Protects against mitochondrial dysfunction during cellular stress, potentially by phosphorylating mitochondrial proteins (By similarity). Plays a role in mitophagy. (641 aa) |
| | F01G4.6 | Phosphate carrier protein, mitochondrial; Transport of phosphate groups from the cytosol to the mitochondrial matrix; Belongs to the mitochondrial carrier (TC 2.A.29) family. (387 aa) |
| | F02A9.10 | Probable methylcrotonoyl-CoA carboxylase beta chain, mitochondrial; Carboxyltransferase subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3- methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism; Belongs to the AccD/PCCB family. (608 aa) |
| | F02A9.10-2 | Ribosomal protein L44, mitochondrial; Component of the mitochondrial ribosome. May have a function in the assembly/stability of nascent mitochondrial polypeptides exiting the ribosome. (394 aa) |
| | clpp-1 | ATP-dependent Clp protease proteolytic subunit 1, mitochondrial; Clp cleaves peptides in various proteins in a process that requires ATP hydrolysis. Clp may be responsible for a fairly general and central housekeeping function rather than for the degradation of specific substrates; Belongs to the peptidase S14 family. (221 aa) |
| | ogdh-2 | Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1 homolog, mitochondrial; The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of three enzymatic components: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3) (By similarity). (911 aa) |
| | dbt-1 | 2-oxoisovalerate dehydrogenase E2 component (dihydrolipoyl transacylase); The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2) (By similarity). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3) (By similarity). Within this complex, the catalytic function of this enzyme is to accept, and to transfer to coenzyme A, acyl groups that are generated by the branched-chain alpha-keto acid decarboxyl [...] (448 aa) |
| | coq-5 | 2-methoxy-6-polyprenyl-1,4-benzoquinol methylase, mitochondrial; Methyltransferase required for the conversion of 2- polyprenyl-6-methoxy-1,4-benzoquinol (DDMQH2) to 2-polyprenyl-3-methyl- 6-methoxy-1,4-benzoquinol (DMQH2). (285 aa) |
| | pdhk-2 | Probable [pyruvate dehydrogenase (acetyl-transferring)] kinase, mitochondrial; Inhibits the mitochondrial pyruvate dehydrogenase complex by phosphorylation of the E1 alpha subunit, thus contributing to the regulation of glucose metabolism. (401 aa) |
| | mmcm-1 | Probable methylmalonyl-CoA mutase, mitochondrial; Involved, in man, in the degradation of several amino acids, odd-chain fatty acids and cholesterol via propionyl-CoA to the tricarboxylic acid cycle. MCM has different functions in other species (By similarity). (744 aa) |
| | mppb-1 | Mitochondrial-processing peptidase subunit beta; Catalytic subunit of the essential mitochondrial processing protease (MPP), which cleaves the mitochondrial sequence off newly imported precursors proteins (Probable). Preferentially, cleaves after an arginine at position P2 (Probable). (458 aa) |
| | clk-1 | 5-demethoxyubiquinone hydroxylase, mitochondrial; Catalyzes the hydroxylation of 2-polyprenyl-3-methyl-6- methoxy-1,4-benzoquinol (DMQH2) during ubiquinone biosynthesis. Has also a structural role in the COQ enzyme complex, stabilizing other COQ polypeptides. Involved in lifespan determination in a ubiquinone- independent manner; Belongs to the COQ7 family. (187 aa) |
| | atfs-1 | Stress activated transcription factor atfs-1; Acts as a transcription factor during mitochondrial stress by activating the mitochondrial unfolded protein response (mtUPR). Induces nuclear and mitochondrial gene transcription, including genes coding for mitochondrial chaperones and proteins involved in glycolysis, amino acid catabolism and innate immunity. Following mitochondrial stress, restores mitochondrial respiratory capacity by limiting the transcription of oxidative phosphorylation (OXPHOS) machinery genes and by promoting the assembly of OXPHOS complexes via the up-regulation of [...] (488 aa) |
| | Y82E9BR.3 | ATP synthase lipid-binding protein, 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 [...] (116 aa) |
| | mppa-1 | Mitochondrial-processing peptidase subunit alpha; Substrate recognition and binding subunit of the essential mitochondrial processing protease (MPP), which cleaves the mitochondrial sequence off newly imported precursors proteins. (514 aa) |
| | Y71F9B.2 | Phosphatidate cytidylyltransferase, mitochondrial; Catalyzes the formation of CDP-diacylglycerol (CDP-DAG) from phosphatidic acid (PA) in the mitochondrial inner membrane. Required for the biosynthesis of the dimeric phospholipid cardiolipin, which stabilizes supercomplexes of the mitochondrial respiratory chain in the mitochondrial inner membrane. (321 aa) |
| | micu-1 | Calcium uptake protein 1 homolog, mitochondrial; Key regulator of mitochondrial calcium uniporter (MCU). (534 aa) |
| | mrps-7 | 28S ribosomal protein S7, mitochondrial; Belongs to the universal ribosomal protein uS7 family. (222 aa) |
| | Y48A6B.9 | Enoyl-[acyl-carrier-protein] reductase, mitochondrial; Catalyzes the NADPH-dependent reduction of trans-2-enoyl thioesters in mitochondrial fatty acid synthesis (fatty acid synthesis type II). Fatty acid chain elongation in mitochondria uses acyl carrier protein (ACP) as an acyl group carrier, but the enzyme accepts both ACP and CoA thioesters as substrates in vitro. Belongs to the zinc-containing alcohol dehydrogenase family. Quinone oxidoreductase subfamily. (346 aa) |
| | Y45G12B.3 | L-2-hydroxyglutarate dehydrogenase, mitochondrial. (433 aa) |
| | Y45F10D.4 | NifU_N domain-containing protein; Scaffold protein for the de novo synthesis of iron-sulfur (Fe-S) clusters within mitochondria, which is required for maturation of both mitochondrial and cytoplasmic [2Fe-2S] and [4Fe-4S] proteins. (153 aa) |
| | bckd-1A | 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) (By similarity). Required for the production of the monomethyl branched-chain fatty acids (mmBCFAs) isopentadecanoate (C15iso) and isoheptadecanoate (C17iso). (432 aa) |
| | Y39B6A.34 | COA8 family protein Y39B6A.34, mitochondrial; May be required for cytochrome c complex (COX) assembly and function, COX being the terminal component of the mitochondrial respiratory chain; Belongs to the COA8 family. (142 aa) |
| | hphd-1 | Hydroxyacid-oxoacid transhydrogenase, mitochondrial; Catalyzes the cofactor-independent reversible oxidation of gamma-hydroxybutyrate (GHB) to succinic semialdehyde (SSA) coupled to reduction of 2-ketoglutarate (2-KG) to D-2-hydroxyglutarate (D-2-HG). L-3-hydroxybutyrate (L-3-OHB) is also a substrate for HOT when using 2- KG as hydrogen acceptor, resulting in the formation of D-2-HG. Belongs to the iron-containing alcohol dehydrogenase family. Hydroxyacid-oxoacid transhydrogenase subfamily. (465 aa) |
| | cox-5A | Cytochrome c oxidase subunit 5A, mitochondrial; Component of the cytochrome c oxidase, the last enzyme in the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol- cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over the inner membrane that drives transmembrane [...] (174 aa) |
| | hsp-60 | Chaperonin homolog Hsp-60, mitochondrial; Implicated in mitochondrial protein import and macromolecular assembly. May facilitate the correct folding of imported proteins. May also prevent misfolding and promote the refolding and proper assembly of unfolded polypeptides generated under stress conditions in the mitochondrial matrix (By similarity). (568 aa) |
| | mrpl-45 | Probable 39S ribosomal protein L45, mitochondrial. (357 aa) |
| | mrpl-19 | Probable 39S ribosomal protein L19, mitochondrial. (288 aa) |
| | nduf-7 | Probable NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, 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). (199 aa) |
| | mecr-1 | Enoyl-[acyl-carrier-protein] reductase, mitochondrial; Catalyzes the NADPH-dependent reduction of trans-2-enoyl thioesters in mitochondrial fatty acid synthesis (fatty acid synthesis type II). Fatty acid chain elongation in mitochondria uses acyl carrier protein (ACP) as an acyl group carrier, but the enzyme accepts both ACP and CoA thioesters as substrates in vitro. Belongs to the zinc-containing alcohol dehydrogenase family. Quinone oxidoreductase subfamily. (344 aa) |
| | immt-2 | MICOS complex subunit MIC60-2; Sustains mitochondrial morphology probably through maintaining cristae morphology. May act as a component of the MICOS complex, a large protein complex of the mitochondria (By similarity). (654 aa) |
| | W03F8.3 | Probable peptide chain release factor 1, mitochondrial; Mitochondrial peptide chain release factor that directs the termination of translation in response to the peptide chain termination codons UAA and UAG. (389 aa) |
| | aars-1 | Alanine--tRNA ligase, 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. (793 aa) |
| | T25G3.4 | Probable glycerol-3-phosphate dehydrogenase, mitochondrial. (722 aa) |
| | ogdh-1 | 2-oxoglutarate dehydrogenase, mitochondrial; The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of three enzymatic components: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3) (By similarity). (1029 aa) |
| | scpl-4 | Mitochondrial import inner membrane translocase subunit TIM50; Essential component of the TIM23 complex, a complex that mediates the translocation of transit peptide-containing proteins across the mitochondrial inner membrane; Belongs to the TIM50 family. (452 aa) |
| | T20H4.5 | Probable NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, 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). (212 aa) |
| | cts-1 | Probable citrate synthase, mitochondrial. (468 aa) |
| | mrrf-1 | Ribosome-recycling factor, mitochondrial; Responsible for the release of ribosomes from messenger RNA at the termination of protein biosynthesis. May increase the efficiency of translation by recycling ribosomes from one round of translation to another (By similarity); Belongs to the RRF family. (238 aa) |
| | immt-1 | MICOS complex subunit MIC60-1; Sustains mitochondrial morphology probably through maintaining cristae morphology. May act as a component of the MICOS complex, a large protein complex of the mitochondria (By similarity). (679 aa) |
| | mrpl-51 | 39S ribosomal protein L51, mitochondrial; Belongs to the mitochondrion-specific ribosomal protein mL51 family. (199 aa) |
| | tin-44 | Probable mitochondrial import inner membrane translocase subunit tin-44; 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. (425 aa) |
| | acdh-10 | Probable medium-chain specific acyl-CoA dehydrogenase 10, mitochondrial; This enzyme is specific for acyl chain lengths of 4 to 16. Belongs to the acyl-CoA dehydrogenase family. (417 aa) |
| | mev-1 | Succinate dehydrogenase cytochrome b560 subunit, mitochondrial; Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q) (By similarity). Mediates resistance to enteropathogenic E.coli infection. Belongs to the cytochrome b560 family. (182 aa) |
| | pdha-1 | Probable pyruvate dehydrogenase E1 component subunit alpha, mitochondrial; The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2). It contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). (414 aa) |
| | tfbm-1 | Dimethyladenosine transferase 1, mitochondrial; Probable S-adenosyl-L-methionine-dependent methyltransferase which specifically dimethylates mitochondrial 12S rRNA at the conserved stem loop. Also required for basal transcription of mitochondrial DNA. Stimulates transcription independently of the methyltransferase activity (By similarity); Belongs to the class I-like SAM-binding methyltransferase superfamily. rRNA adenine N(6)-methyltransferase family. KsgA subfamily. (367 aa) |
| | idhg-2 | Isocitrate dehydrogenase [NAD] subunit, mitochondrial. (373 aa) |
| | mmaa-1 | Methylmalonic aciduria type A homolog, mitochondrial; May have GTPase activity. May also bind and hydrolyze ATP. May function as chaperone (By similarity). Likely to have a role in propionyl-CoA metabolism and adenosylcobalamin synthesis. (399 aa) |
| | gldc-1 | Glycine cleavage system P protein; The glycine cleavage system catalyzes the degradation of glycine. (979 aa) |
| | R05D3.12 | Putative DNA topoisomerase 2, mitochondrial; Control of topological states of DNA by transient breakage and subsequent rejoining of DNA strands. Topoisomerase II makes double- strand breaks (By similarity); Belongs to the type II topoisomerase family. (1170 aa) |
| | F54D5.7 | Probable glutaryl-CoA dehydrogenase, mitochondrial. (409 aa) |
| | F53F4.10 | Probable NADH dehydrogenase [ubiquinone] flavoprotein 2, 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). (239 aa) |
| | gcsh-2 | Glycine cleavage system H protein; The H protein shuttles the methylamine group of glycine from the P protein to the T protein; Belongs to the GcvH family. (146 aa) |
| | suca-1 | Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial; ATP-specific succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of ATP and thus represents the only step of substrate-level phosphorylation in the TCA. The beta subunit provides nucleotide specificity of the enzyme and binds the substrate succinate, while the binding sites for coenzyme A and phosphate are found in the alpha subunit. (435 aa) |
| | F45G2.9 | rRNA methyltransferase 2, mitochondrial; S-adenosyl-L-methionine-dependent 2'-O-ribose methyltransferase that catalyzes the formation of 2'-O-methyluridine at position 808 (Um808) in the mitochondrial large subunit ribosomal RNA (mtLSU rRNA), a universally conserved modification in the peptidyl transferase domain of the mtLSU rRNA. (214 aa) |
| | F44G4.2 | Probable NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial; 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 (By similarity). (160 aa) |
| | idha-1 | Probable isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial; Belongs to the isocitrate and isopropylmalate dehydrogenases family. (358 aa) |
| | sdhb-1 | Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial; Iron-sulfur protein (IP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). (298 aa) |
| | idhg-1 | Isocitrate dehydrogenase [NAD] subunit, mitochondrial. (396 aa) |
| | mrps-24 | 28S ribosomal protein S24, mitochondrial; Belongs to the universal ribosomal protein uS3 family. (164 aa) |
| | sdhd-1 | Putative succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial; Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). (145 aa) |
| | gfm-1 | Elongation factor G, mitochondrial; Mitochondrial GTPase that catalyzes the GTP-dependent ribosomal translocation step during translation elongation. During this step, the ribosome changes from the pre-translocational (PRE) to the post-translocational (POST) state as the newly formed A-site-bound peptidyl-tRNA and P-site-bound deacylated tRNA move to the P and E sites, respectively. Catalyzes the coordinated movement of the two tRNA molecules, the mRNA and conformational changes in the ribosome. (750 aa) |
| | pcca-1 | Propionyl-CoA carboxylase alpha chain, mitochondrial; This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites. Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl- CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA (By similarity). Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl car [...] (724 aa) |
| | F27D4.1 | Probable electron transfer flavoprotein subunit alpha, mitochondrial; The electron transfer flavoprotein serves as a specific electron acceptor for several dehydrogenases, including five acyl-CoA dehydrogenases, glutaryl-CoA and sarcosine dehydrogenase. It transfers the electrons to the main mitochondrial respiratory chain via ETF- ubiquinone oxidoreductase (ETF dehydrogenase) (By similarity). (332 aa) |
| | gcst-1 | Aminomethyltransferase; The glycine cleavage system catalyzes the degradation of glycine; Belongs to the GcvT family. (402 aa) |
| | dlat-1 | Pyruvate dehydrogenase E2 component (dihydrolipoamide acetyltransferase); The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2). It contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). (507 aa) |
| | nduf-6 | Probable NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial; 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 (By similarity). (140 aa) |
| | mdh-2 | Probable malate dehydrogenase, mitochondrial. (341 aa) |
| | emre-1 | Essential MCU regulator, mitochondrial; Essential regulatory subunit of the mitochondrial calcium uniporter (mcu-1) channel, a protein that mediates calcium uptake into mitochondria; Belongs to the SMDT1/EMRE family. (90 aa) |
| | F13E9.13 | Uncharacterized protein F13E9.13, mitochondrial. (277 aa) |
| | alh-8 | Probable methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial; Plays a role in valine and pyrimidine metabolism. Binds fatty acyl-CoA (By similarity); Belongs to the aldehyde dehydrogenase family. (523 aa) |
| | mrpl-11 | Probable 39S ribosomal protein L11, mitochondrial; Belongs to the universal ribosomal protein uL11 family. (195 aa) |
| | psd-1 | Phosphatidylserine decarboxylase proenzyme, mitochondrial; Catalyzes the formation of phosphatidylethanolamine (PtdEtn) from phosphatidylserine (PtdSer). Plays a central role in phospholipid metabolism and in the interorganelle trafficking of phosphatidylserine. (377 aa) |
| | mrpl-41 | 39S ribosomal protein L41, mitochondrial; Belongs to the mitochondrion-specific ribosomal protein mL41 family. (180 aa) |
| | prdh-1 | Proline dehydrogenase 1, mitochondrial; Converts proline to delta-1-pyrroline-5-carboxylate (By similarity). Through proline catabolism, promotes reactive oxygen species (ROS) production and the transcription of skn-1 target genes in response to bacterial infection by P.aeruginosa. (616 aa) |
| | mai-2 | ATPase inhibitor mai-2, mitochondrial; Thought to be a regulatory component of the ATP-synthesizing complex in the mitochondria. Activity is pH dependent. (109 aa) |
| | C01G10.10 | Probable tRNA N6-adenosine threonylcarbamoyltransferase, mitochondrial; Required for the formation of a threonylcarbamoyl group on adenosine at position 37 (t(6)A37) in mitochondrial tRNAs that read codons beginning with adenine. Probably involved in the transfer of the threonylcarbamoyl moiety of threonylcarbamoyl-AMP (TC-AMP) to the N6 group of A37 (By similarity). Involved in mitochondrial genome maintenance; Belongs to the KAE1 / TsaD family. (421 aa) |
| | sdha-1 | Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial; Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q); Belongs to the FAD-dependent oxidoreductase 2 family. FRD/SDH subfamily. (646 aa) |
| | pdhb-1 | Pyruvate dehydrogenase E1 component subunit beta, mitochondrial; The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2). It contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3) (By similarity). (352 aa) |
| | C05C10.3 | Probable succinyl-CoA:3-ketoacid coenzyme A transferase, mitochondrial; Key enzyme for ketone body catabolism. Transfers the CoA moiety from succinate to acetoacetate. Formation of the enzyme-CoA intermediate proceeds via an unstable anhydride species formed between the carboxylate groups of the enzyme and substrate. (521 aa) |
| | let-721 | Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial; Accepts electrons from ETF and reduces ubiquinone. Belongs to the ETF-QO/FixC family. (597 aa) |
| | sucl-1 | Succinate--CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial; Succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of either ATP or GTP and thus represents the only step of substrate-level phosphorylation in the TCA. The alpha subunit of the enzyme binds the substrates coenzyme A and phosphate, while succinate binding and specificity for either ATP or GTP is provided by different beta subunits. (322 aa) |
| | sod-3 | Superoxide dismutase [Mn] 2, mitochondrial; Destroys superoxide anion radicals which are normally produced within the cells and which are toxic to biological systems. (218 aa) |
| | C08F8.2 | ATP-dependent RNA helicase SUV3 homolog, mitochondrial; ATPase and DNA/RNA helicase able to unwind DNA/DNA, DNA/RNA and RNA/RNA duplexes in the 5'-3' direction. (719 aa) |
| | nuo-1 | NADH dehydrogenase [ubiquinone] flavoprotein 1, 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. (479 aa) |
| | ncx-9 | Mitochondrial sodium/calcium exchanger protein; Mitochondrial sodium/calcium antiporter that mediates sodium- dependent calcium efflux from mitochondrion, thereby acting as a key regulator of mitochondrion calcium homeostasis (Probable). Required for patterning of neural circuits: functions in the same pathway as RAC- dependent effectors of the unc-6/netrin signaling pathway to set left/ right patterning of the VD/DD GABAergic circuit. Belongs to the Ca(2+):cation antiporter (CaCA) (TC 2.A.19) family. SLC24A subfamily. (701 aa) |
| | oatr-1 | Probable ornithine aminotransferase, mitochondrial; Belongs to the class-III pyridoxal-phosphate-dependent aminotransferase family. (422 aa) |
| | mrpl-32 | Probable 39S ribosomal protein L32, mitochondrial. (183 aa) |
| | mtss-1 | Single-stranded DNA-binding protein, mitochondrial; Has a role in germline cell development and proliferation which affects the frequency of apoptosis. Required for mitochondrial DNA replication and transcription and fertility. (170 aa) |
| | mrpl-35 | Probable 39S ribosomal protein L35, mitochondrial. (158 aa) |
| | dld-1 | Dihydrolipoyl dehydrogenase, mitochondrial. (495 aa) |
| | K11H3.3 | Putative tricarboxylate transport protein, mitochondrial; Transport of citrate across inner mitochondrial membrane. Belongs to the mitochondrial carrier (TC 2.A.29) family. (312 aa) |
| | mai-1 | ATPase inhibitor mai-1, mitochondrial; Thought to be a regulatory component of the ATP-synthesizing complex in the mitochondria. (88 aa) |
| | gas-1 | Probable NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, 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). (482 aa) |
| | mrps-15 | 28S ribosomal protein S15, mitochondrial; Belongs to the universal ribosomal protein uS15 family. (330 aa) |
| | gta-1 | Probable 4-aminobutyrate aminotransferase, mitochondrial. (483 aa) |
| | moma-1 | MICOS complex subunit MIC27; Sustains mitochondrial morphology probably through maintaining cristae morphology. May act as a component of the MICOS complex, a large protein complex of the mitochondria (By similarity). (201 aa) |
| | mcu-1 | Calcium uniporter protein, mitochondrial; Mitochondrial inner membrane calcium uniporter that mediates calcium uptake into mitochondria. Constitutes a pore- forming and calcium-conducting subunit (By similarity). Mitochondrial calcium homeostasis plays key roles in cellular physiology and regulates cell bioenergetics, cytoplasmic calcium signals and activation of cell death pathways (By similarity). Required for rapid mitochondrial calcium uptake and mitochondrial reactive oxygen species (mtROS) production after wounding. (333 aa) |
| | atp-1 | ATP synthase subunit alpha, 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 [...] (538 aa) |
| | fum-1 | Probable fumarate hydratase, mitochondrial; Catalyzes the reversible stereospecific interconversion of fumarate to L-malate. In mitochondrion, catalyzes the hydration of fumarate to L-malate in the tricarboxylic acid (TCA) cycle to facilitate a transition step in the production of energy in the form of NADH; Belongs to the class-II fumarase/aspartase family. Fumarase subfamily. (501 aa) |
| | frh-1 | Frataxin, mitochondrial; Promotes the biosynthesis of heme as well as the assembly and repair of iron-sulfur clusters by delivering Fe(2+) to proteins involved in these pathways. May play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe(2+) to Fe(3+). May be able to store large amounts of the metal in the form of a ferrihydrite mineral by oligomerization. Belongs to the frataxin family. (136 aa) |
| | mrpl-24 | Probable 39S ribosomal protein L24, mitochondrial. (278 aa) |
| | cri-3 | Conserved regulator of innate immunity protein 3; Belongs to the MAM33 family. (236 aa) |
| | F58F12.1 | ATP synthase subunit delta, 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 turnover in the catalytic domain of F(1) is coupled via a rotary mechanism of the c [...] (163 aa) |
| | coq-2 | 4-hydroxybenzoate polyprenyltransferase, mitochondrial; Catalyzes the prenylation of para-hydroxybenzoate (PHB) with an all-trans polyprenyl group. Mediates the second step in the final reaction sequence of coenzyme Q (CoQ) biosynthesis, which is the condensation of the polyisoprenoid side chain with PHB, generating the first membrane-bound Q intermediate; Belongs to the UbiA prenyltransferase family. (370 aa) |
| | rad-8 | Reticulon-4-interacting protein 1, mitochondrial; Plays a role in oxygen metabolism in the mitochondria by regulating the levels of reactive oxygen species (ROS) thereby conferring resistance to oxidative stress. Involved in resistance to P.aeruginosa PA14 infection. Regulates lifespan. Belongs to the zinc-containing alcohol dehydrogenase family. Quinone oxidoreductase subfamily. (366 aa) |
| | aco-2 | Probable aconitate hydratase, mitochondrial; Catalyzes the isomerization of citrate to isocitrate via cis- aconitate. (777 aa) |
| | cox-6A | Cytochrome c oxidase subunit 6A, mitochondrial; Component of the cytochrome c oxidase, the last enzyme in the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol- cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over the inner membrane that drives transmembrane [...] (128 aa) |
