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| PYCRL | Pyrroline-5-carboxylate reductase 3; Enzyme that catalyzes the last step in proline biosynthesis. Proline is synthesized from either glutamate or ornithine; both are converted to pyrroline-5-carboxylate (P5C), and then to proline via pyrroline-5-carboxylate reductases (PYCRs). PYCRL is exclusively linked to the conversion of ornithine to proline (286 aa) | |||
| DHDH | Dihydrodiol dehydrogenase; Belongs to the Gfo/Idh/MocA family (334 aa) | |||
| TGDS | dTDP-D-glucose 4,6-dehydratase; Short chain dehydrogenase/reductase superfamily (350 aa) | |||
| BBOX1 | Gamma-butyrobetaine dioxygenase; Catalyzes the formation of L-carnitine from gamma- butyrobetaine; Belongs to the gamma-BBH/TMLD family (387 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 (725 aa) | |||
| GFOD2 | Glucose-fructose oxidoreductase domain-containing protein 2; Promotes matrix assembly (385 aa) | |||
| NOS3 | Nitric oxide synthase, endothelial; Produces nitric oxide (NO) which is implicated in vascular smooth muscle relaxation through a cGMP-mediated signal transduction pathway. NO mediates vascular endothelial growth factor (VEGF)-induced angiogenesis in coronary vessels and promotes blood clotting through the activation of platelets (1203 aa) | |||
| UQCRFS1 | Cytochrome b-c1 complex subunit Rieske, mitochondrial; Cytochrome b-c1 complex subunit Rieske, mitochondrial- Component of the mitochondrial ubiquinol-cytochrome c reductase complex dimer (complex III dimer), which is a respiratory chain that generates an electrochemical potential coupled to ATP synthesis. Incorporation of UQCRFS1 is the penultimate step in complex III assembly (By similarity) (274 aa) | |||
| BCAT2 | Branched-chain-amino-acid aminotransferase, mitochondrial; Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine. May also function as a transporter of branched chain alpha-keto acids; Belongs to the class-IV pyridoxal-phosphate-dependent aminotransferase family (392 aa) | |||
| CNDP2 | Cytosolic non-specific dipeptidase; Hydrolyzes a variety of dipeptides including L-carnosine but has a strong preference for Cys-Gly. Acts as a functional tumor suppressor in gastric cancer via activation of the mitogen-activated protein kinase (MAPK) pathway. An elevated level of CNDP2 activates the p38 and JNK MAPK pathways to induce cell apoptosis, and a lower level of CNDP2 activates the ERK MAPK pathway to promote cell proliferation. Isoform 2 may play a role as tumor suppressor in hepatocellular carcinoma (HCC) cells. Catalyzes the production of N- lactoyl-amino acids from lactat [...] (475 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 (By similarity). As component of the iNOS-S100A8/9 transnitrosylase complex involved in the selective inflammatory stimulus-dependent S-nitrosylation of GAPDH on ’Cys-247’ implicated in regulation of the GAIT complex activity and probably multiple targets including ANXA5, EZR [...] (1153 aa) | |||
| TMLHE | Trimethyllysine dioxygenase, mitochondrial; Converts trimethyllysine (TML) into hydroxytrimethyllysine (HTML); Belongs to the gamma-BBH/TMLD family (421 aa) | |||
| PYCR2 | Pyrroline-5-carboxylate reductase 2; Housekeeping enzyme that catalyzes the last step in proline biosynthesis. In some cell types, such as erythrocytes, its primary function may be the generation of NADP(+). Can utilize both NAD and NADP. Has higher affinity for NADP, but higher catalytic efficiency with NADH. Involved in cellular response to oxidative stress (320 aa) | |||
| CNDP1 | Carnosine dipeptidase 1; Belongs to the peptidase M20A family (507 aa) | |||
| PM20D1 | N-fatty-acyl-amino acid synthase/hydrolase PM20D1; Bidirectional N-fatty-acyl amino acid synthase/hydrolase that regulates the production of N-fatty-acyl amino acids. These metabolites are endogenous chemical uncouplers of mitochondrial respiration. In an UCP1-independent manner, maybe through interaction with mitochondrial transporters, they promote proton leakage into the mitochondrial matrix. Thereby, this secreted protein may indirectly regulate the bodily dissipation of chemical energy as heat through thermogenic respiration (502 aa) | |||
| ALDH4A1 | Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial; Irreversible conversion of delta-1-pyrroline-5- carboxylate (P5C), derived either from proline or ornithine, to glutamate. This is a necessary step in the pathway interconnecting the urea and tricarboxylic acid cycles. The preferred substrate is glutamic gamma-semialdehyde, other substrates include succinic, glutaric and adipic semialdehydes (563 aa) | |||
| GFOD1 | Glucose-fructose oxidoreductase domain containing 1 (390 aa) | |||
| NOXRED1 | NADP-dependent oxidoreductase domain-containing protein 1; Probable oxidoreductase (359 aa) | |||
| ACY1 | Aminoacylase-1; Involved in the hydrolysis of N-acylated or N-acetylated amino acids (except L-aspartate); Belongs to the peptidase M20A family (408 aa) | |||
| PYCR1 | Pyrroline-5-carboxylate reductase 1, mitochondrial; Housekeeping enzyme that catalyzes the last step in proline biosynthesis. Can utilize both NAD and NADP, but has higher affinity for NAD. Involved in the cellular response to oxidative stress (346 aa) | |||
| BLVRA | Biliverdin reductase A; Reduces the gamma-methene bridge of the open tetrapyrrole, biliverdin IX alpha, to bilirubin with the concomitant oxidation of a NADH or NADPH cofactor; Belongs to the Gfo/Idh/MocA family. Biliverdin reductase subfamily (296 aa) | |||
| ENSG00000255835 | Uncharacterized protein (359 aa) | |||
| POR | NADPH--cytochrome P450 reductase; This enzyme is required for electron transfer from NADP to cytochrome P450 in microsomes. It can also provide electron transfer to heme oxygenase and cytochrome B5; Belongs to the NADPH--cytochrome P450 reductase family (680 aa) | |||
| ENSG00000114786 | ABHD14A-ACY1 readthrough (509 aa) | |||
| NOS1 | Nitric oxide synthase, brain; Produces nitric oxide (NO) which is a messenger molecule with diverse functions throughout the body. In the brain and peripheral nervous system, NO displays many properties of a neurotransmitter. Probably has nitrosylase activity and mediates cysteine S-nitrosylation of cytoplasmic target proteins such SRR; Belongs to the NOS family (1468 aa) | |||
| PIG6 | Proline dehydrogenase 1, mitochondrial; Converts proline to delta-1-pyrroline-5-carboxylate; Belongs to the proline oxidase family (600 aa) | |||
