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Ddo | RCG38204, isoform CRA_d. (341 aa) | ||||
Ube2d1 | Ubiquitin-conjugating enzyme E2 D1; Accepts ubiquitin from the E1 complex and catalyzes its covalent attachment to other proteins. In vitro catalyzes 'Lys-48'- linked polyubiquitination. Mediates the selective degradation of short- lived and abnormal proteins. Functions in the E6/E6-AP-induced ubiquitination of p53/TP53. Mediates auto-ubiquitination of STUB1, TRAF6 and TRIM63/MURF1. Ubiquitinates STUB1-associated HSP90AB1 in vitro. Lacks inherent specificity for any particular lysine residue of ubiquitin. Essential for viral activation of IRF3. Mediates polyubiquitination of CYP3A4 (By [...] (147 aa) | ||||
Agps | Alkyldihydroxyacetonephosphate synthase, peroxisomal; Catalyzes the exchange of the acyl chain in acyl- dihydroxyacetonephosphate (acyl-DHAP) for a long chain fatty alcohol, yielding the first ether linked intermediate, i.e. alkyl- dihydroxyacetonephosphate (alkyl-DHAP), in the pathway of ether lipid biosynthesis; Belongs to the FAD-binding oxidoreductase/transferase type 4 family. (644 aa) | ||||
Ehhadh | Enoyl-CoA hydratase/3,2-trans-enoyl-CoA isomerase; In the N-terminal section; belongs to the enoyl-CoA hydratase/isomerase family. (722 aa) | ||||
Rps27a | Ubiquitin-40S ribosomal protein S27a; [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 i [...] (156 aa) | ||||
Hao1 | Hydroxyacid oxidase 1 (Mapped). (370 aa) | ||||
Baat | Bile acid-CoA:amino acid N-acyltransferase; Catalyzes the amidation of bile acids (BAs) with the amino acids taurine and glycine. More efficient at taurine conjugation of cholyl CoA than glycine conjugation. Amidation of BAs in the liver with glycine or taurine prior to their excretion into bile is an important biochemical event in bile acid metabolism (By similarity). This conjugation (or amidation) plays several important biological roles in that it promotes the secretion of BAs and cholesterol into bile and increases the detergent properties of BAs in the intestine, which facilitate [...] (420 aa) | ||||
Acox2 | Peroxisomal acyl-coenzyme A oxidase 2; Oxidizes the CoA esters of the bile acid intermediates di- and tri-hydroxycoprostanic acids. Capable of oxidizing short as well as long chain 2-methyl branched fatty acids. (681 aa) | ||||
Cat | Catalase; Occurs in almost all aerobically respiring organisms and serves to protect cells from the toxic effects of hydrogen peroxide. Promotes growth of cells; Belongs to the catalase family. (527 aa) | ||||
Pex2 | Peroxisome biogenesis factor 2; Somewhat implicated in the biogenesis of peroxisomes; Belongs to the pex2/pex10/pex12 family. (305 aa) | ||||
Pipox | Pipecolic acid and sarcosine oxidase. (390 aa) | ||||
Hmgcl | Hydroxymethylglutaryl-CoA lyase, mitochondrial; Mitochondrial 3-hydroxymethyl-3-methylglutaryl-CoA lyase that catalyzes a cation-dependent cleavage of (S)-3-hydroxy-3- methylglutaryl-CoA into acetyl-CoA and acetoacetate, a key step in ketogenesis. Terminal step in leucine catabolism. Ketone bodies (beta- hydroxybutyrate, acetoacetate and acetone) are essential as an alternative source of energy to glucose, as lipid precursors and as regulators of metabolism; Belongs to the HMG-CoA lyase family. (325 aa) | ||||
Pex12 | Peroxisome assembly protein 12; Required for protein import into peroxisomes. (359 aa) | ||||
Acot2 | Acyl-coenzyme A thioesterase 2, mitochondrial; Acyl-CoA thioesterases are a group of enzymes that catalyze the hydrolysis of acyl-CoAs to the free fatty acid and coenzyme A (CoASH), providing the potential to regulate intracellular levels of acyl-CoAs, free fatty acids and CoASH (By similarity). Acyl-coenzyme A thioesterase 2/ACOT2 displays higher activity toward long chain acyl CoAs (C14-C20). The enzyme is involved in enhancing the hepatic fatty acid oxidation in mitochondria (By similarity). Belongs to the C/M/P thioester hydrolase family. (453 aa) | ||||
Acot4 | Acyl-CoA thioesterase 4. (421 aa) | ||||
Acot5 | Acyl-CoA thioesterase 5. (421 aa) | ||||
Scp2 | Non-specific lipid-transfer protein; Mediates in vitro the transfer of all common phospholipids, cholesterol and gangliosides between membranes. May play a role in regulating steroidogenesis. (643 aa) | ||||
Pex7 | Peroxisomal biogenesis factor 7. (318 aa) | ||||
Zfand6 | AN1-type zinc finger protein 6; Involved in regulation of TNF-alpha induced NF-kappa-B activation and apoptosis. Involved in modulation of 'Lys-48'-linked polyubiquitination status of TRAF2 and decreases association of TRAF2 with RIPK1. Required for PTS1 target sequence-dependent protein import into peroxisomes and PEX5 stability; may cooperate with PEX6. In vitro involved in PEX5 export from the cytosol to peroxisomes (By similarity). (223 aa) | ||||
Pex14 | Peroxisomal membrane protein PEX14; Peroxisome membrane protein that is an essential component of the peroxisomal import machinery. Functions as a docking factor for the predominantly cytoplasmic PTS1 receptor (PEX5). Plays a key role for peroxisome movement through a direct interaction with tubulin. (376 aa) | ||||
Mlycd | Malonyl-CoA decarboxylase, mitochondrial; Catalyzes the conversion of malonyl-CoA to acetyl-CoA. In the fatty acid biosynthesis MCD selectively removes malonyl-CoA and thus assures that methyl-malonyl-CoA is the only chain elongating substrate for fatty acid synthase and that fatty acids with multiple methyl side chains are produced. In peroxisomes it may be involved in degrading intraperoxisomal malonyl-CoA, which is generated by the peroxisomal beta-oxidation of odd chain-length dicarboxylic fatty acids. Plays a role in the metabolic balance between glucose and lipid oxidation in mus [...] (492 aa) | ||||
Idh1 | Isocitrate dehydrogenase [NADP] cytoplasmic. (414 aa) | ||||
Acot8 | Acyl-coenzyme A thioesterase 8; Acyl-coenzyme A (acyl-CoA) thioesterases are a group of enzymes that catalyze the hydrolysis of acyl-CoAs to the free fatty acid and coenzyme A (CoASH), providing the potential to regulate intracellular levels of acyl-CoAs, free fatty acids and CoASH. Acyl-coenzyme A thioesterase 8/ACOT8 display no strong substrate specificity with respect to the carboxylic acid moiety of Acyl-CoAs. Hydrolyzes medium length (C2 to C20) straight-chain, saturated and unsaturated acyl-CoAS but is inactive towards substrates with longer aliphatic chains. Moreover, it catalyz [...] (374 aa) | ||||
Lonp2 | Lon protease homolog 2, peroxisomal; ATP-dependent serine protease that mediates the selective degradation of misfolded and unassembled polypeptides in the peroxisomal matrix. Necessary for type 2 peroxisome targeting signal (PTS2)-containing protein processing and facilitates peroxisome matrix protein import. May indirectly regulate peroxisomal fatty acid beta- oxidation through degradation of the self-processed forms of TYSND1. Belongs to the peptidase S16 family. (852 aa) | ||||
Eci2 | Enoyl-CoA delta isomerase 2, mitochondrial; Able to isomerize both 3-cis and 3-trans double bonds into the 2-trans form in a range of enoyl-CoA species. Has a preference for 3-trans substrates. (391 aa) | ||||
Gstk1 | Glutathione S-transferase kappa 1; Might confer protection against genotoxic and cytotoxic electrophiles in the mitochondrial compartment; Belongs to the GST superfamily. Kappa family. (226 aa) | ||||
Pex6 | Peroxisome assembly factor 2; Involved in peroxisome biosynthesis. Required for stability of the PTS1 receptor. Probably required for protein import into peroxisomes. Anchored by PEX26 to peroxisome membranes, possibly to form heteromeric AAA ATPase complexes required for the import of proteins into peroxisomes (By similarity). (978 aa) | ||||
Ide | Insulin-degrading enzyme; Plays a role in the cellular breakdown of insulin, APP peptides, IAPP peptides, glucagon, bradykinin, kallidin and other peptides, and thereby plays a role in intercellular peptide signaling. Substrate binding induces important conformation changes, making it possible to bind and degrade larger substrates, such as insulin (By similarity). Contributes to the regulation of peptide hormone signaling cascades and regulation of blood glucose homeostasis via its role in the degradation of insulin, glucagon and IAPP (By similarity). Plays a role in the degradation an [...] (1019 aa) | ||||
Ephx2 | Bifunctional epoxide hydrolase 2; Bifunctional enzyme. The C- terminal domain has epoxide hydrolase activity and acts on epoxides (alkene oxides, oxiranes) and arene oxides. Plays a role in xenobiotic metabolism by degrading potentially toxic epoxides. Also determines steady-state levels of physiological mediators (By similarity). The N-terminal domain has lipid phosphatase activity, with the highest activity towards threo-9,10-phosphonooxy- hydroxy-octadecanoic acid, followed by erythro-9,10-phosphonooxy- hydroxy-octadecanoic acid, 12-phosphonooxy-octadec-9Z-enoic acid and 12-phosphon [...] (554 aa) | ||||
Phyh | Phytanoyl-CoA dioxygenase, peroxisomal; Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA. (338 aa) | ||||
Dhrs4 | Dehydrogenase/reductase SDR family member 4; Reduces all-trans-retinal and 9-cis retinal. Can also catalyze the oxidation of all-trans-retinol with NADP as co-factor, but with much lower efficiency. Reduces alkyl phenyl ketones and alpha- dicarbonyl compounds with aromatic rings, such as pyrimidine-4- aldehyde, 3-benzoylpyridine, 4-benzoylpyridine, menadione and 4- hexanoylpyridine. Has no activity towards aliphatic aldehydes and ketones (By similarity). (279 aa) | ||||
Hacl1 | 2-hydroxyacyl-CoA lyase 1; Catalyzes a carbon-carbon cleavage reaction; cleaves a 2- hydroxy-3-methylacyl-CoA into formyl-CoA and a 2-methyl-branched fatty aldehyde; Belongs to the TPP enzyme family. (581 aa) | ||||
Gnpat | Dihydroxyacetone phosphate acyltransferase. (678 aa) | ||||
Uba52 | Ubiquitin-60S ribosomal protein L40; [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 in [...] (128 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; Belongs to the enoyl-CoA hydratase/isomerase family. (327 aa) | ||||
Agxt | Serine--pyruvate aminotransferase, mitochondrial; Dual metabolic roles of gluconeogenesis (in the mitochondria) and glyoxylate detoxification (in the peroxisomes). (414 aa) | ||||
Pex10 | Peroxisomal biogenesis factor 10. (324 aa) | ||||
Pex1 | Similar to peroxisome biogenesis factor 1 (Predicted). (1283 aa) | ||||
Nudt7 | Nudix (Nucleoside diphosphate linked moiety X)-type motif 7 (Predicted), isoform CRA_d. (260 aa) | ||||
Tysnd1 | Trypsin domain containing 1 (Predicted), isoform CRA_a. (567 aa) | ||||
Mpv17 | Protein Mpv17; Non-selective channel that modulates the membrane potential under normal conditions and oxidative stress, and is involved in mitochondrial homeostasis. Involved in mitochondrial deoxynucleoside triphosphates (dNTP) pool homeostasis and mitochondrial DNA (mtDNA) maintenance (By similarity). May be involved in the regulation of reactive oxygen species metabolism and the control of oxidative phosphorylation (By similarity); Belongs to the peroxisomal membrane protein PXMP2/4 family. (169 aa) | ||||
Amacr | Alpha-methylacyl-CoA racemase; Catalyzes the interconversion of (R)- and (S)-stereoisomers of alpha-methyl-branched-chain fatty acyl-CoA esters. Acts only on coenzyme A thioesters, not on free fatty acids, and accepts as substrates a wide range of alpha-methylacyl-CoAs, including pristanoyl-CoA, trihydroxycoprostanoyl-CoA (an intermediate in bile acid synthesis), and arylpropionic acids like the anti- inflammatory drug ibuprofen (2-(4-isobutylphenyl)propionic acid) but neither 3-methyl-branched nor linear-chain acyl-CoAs. (454 aa) | ||||
Acox3 | Peroxisomal acyl-coenzyme A oxidase 3; Oxidizes the CoA-esters of 2-methyl-branched fatty acids. Belongs to the acyl-CoA oxidase family. (699 aa) | ||||
Acox1 | Peroxisomal acyl-CoA oxidase 1, A chain; Catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl-CoAs. Isoform 1 shows highest activity against medium-chain fatty acyl-CoAs and activity decreases with increasing chain length. Isoform 2 is active against a much broader range of substrates and shows activity towards very long-chain acyl-CoAs. Isoform 1 shows optimum activity with a chain length of 10 carbons while isoform 2 exhibits optimum activity with 14 carbons. (660 aa) | ||||
Slc27a2 | Very long-chain acyl-CoA synthetase; Acyl CoA synthetase that activates long-chain and very long- chain fatty acids (VLCFAs) by catalyzing the formation of fatty acyl- CoA. Can also activate branched-chain fatty acids such as phytanic acid and pristanic acid (By similarity). Does not activate C24 bile acids, cholate and chenodeoxycholate (By similarity). In vitro, activates 3-alpha,7-alpha,12-alpha- trihydroxy- 5-beta-cholestanate (THCA), the C27 precursor of cholic acid deriving from the de novo synthesis from cholesterol (By similarity). Exhibits long-chain fatty acids (LCFA) transpo [...] (618 aa) | ||||
Ube2d3 | Ubiquitin-conjugating enzyme E2 D3; Accepts ubiquitin from the E1 complex and catalyzes its covalent attachment to other proteins. In vitro catalyzes 'Lys-11'-, as well as 'Lys-48'-linked polyubiquitination. Cooperates with the E2 CDC34 and the SCF(FBXW11) E3 ligase complex for the polyubiquitination of NFKBIA leading to its subsequent proteasomal degradation. Acts as an initiator E2, priming the phosphorylated NFKBIA target at positions 'Lys-21' and/or 'Lys-22' with a monoubiquitin. Ubiquitin chain elongation is then performed by CDC34, building ubiquitin chains from the UBE2D3-primed [...] (147 aa) | ||||
Paox | RCG47968, isoform CRA_a. (531 aa) | ||||
Crat | Carnitine O-acetyltransferase; Catalyzes the reversible transfer of acyl groups from carnitine to coenzyme A (CoA) and regulates the acyl-CoA/CoA ratio. Also plays a crucial role in the transport of fatty acids for beta- oxidation. May be specific for short chain fatty acids. (626 aa) | ||||
Pex26 | Similar to peroxisome biogenesis factor 26. (301 aa) | ||||
Nos2 | Nitric oxide synthase, inducible; Produces nitric oxide (NO) which is a messenger molecule with diverse functions throughout the body. In macrophages, NO mediates tumoricidal and bactericidal actions. Also has nitrosylase activity and mediates cysteine S-nitrosylation of cytoplasmic target proteins such PTGS2/COX2. As component of the iNOS-S100A8/9 transnitrosylase complex involved in the selective inflammatory stimulus-dependent S- nitrosylation of GAPDH implicated in regulation of the GAIT complex activity and probably multiple targets including ANXA5, EZR, MSN and VIM. Involved in i [...] (1147 aa) | ||||
Pex13 | Peroxisomal biogenesis factor 13 (Predicted). (403 aa) | ||||
Dao | D-amino-acid oxidase; Regulates the level of the neuromodulator D-serine in the brain. Has high activity towards D-DOPA and contributes to dopamine synthesis. Could act as a detoxifying agent which removes D-amino acids accumulated during aging. Acts on a variety of D-amino acids with a preference for those having small hydrophobic side chains followed by those bearing polar, aromatic, and basic groups. Does not act on acidic amino acids. (346 aa) | ||||
Pecr | Peroxisomal trans-2-enoyl-CoA reductase; Participates in chain elongation of fatty acids. Has no 2,4- dienoyl-CoA reductase activity (By similarity). (305 aa) | ||||
LOC103690024 | Peroxisomal targeting signal 1 receptor; Binds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import. (640 aa) | ||||
Hsd17b4 | Peroxisomal multifunctional enzyme type 2; Bifunctional enzyme acting on the peroxisomal beta-oxidation pathway for fatty acids. Catalyzes the formation of 3-ketoacyl-CoA intermediates from both straight-chain and 2-methyl-branched-chain fatty acids; Belongs to the short-chain dehydrogenases/reductases (SDR) family. (751 aa) | ||||
Usp9x | Ubiquitin-specific peptidase 9, X-linked; Belongs to the peptidase C19 family. (2554 aa) | ||||
Acot3 | Acyl-CoA thioesterase 3. (429 aa) | ||||
Crot | Peroxisomal carnitine O-octanoyltransferase; Beta-oxidation of fatty acids. The highest activity concerns the C6 to C10 chain length substrate; Belongs to the carnitine/choline acetyltransferase family. (612 aa) | ||||
Hao2 | Hydroxyacid oxidase 2; Catalyzes the oxidation of L-alpha-hydroxy acids as well as, more slowly, that of L-alpha-amino acids. (396 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 [...] (810 aa) | ||||
Nudt19 | Nucleoside diphosphate-linked moiety X motif 19; Acyl-CoA diphosphatase that mediates the hydrolysis of a wide range of CoA esters, including choloyl-CoA and branched-chain fatty- acyl-CoA esters. At low substrate concentrations medium and long-chain fatty-acyl-CoA esters are the primary substrates (By similarity). (357 aa) |