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DLD | Dihydrolipoyl dehydrogenase, mitochondrial; Lipoamide dehydrogenase is a component of the glycine cleavage system as well as an E3 component of three alpha-ketoacid dehydrogenase complexes (pyruvate-, alpha-ketoglutarate-, and branched-chain amino acid-dehydrogenase complex). In monomeric form has additional moonlighting function as serine protease. Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction (By similarity) (509 aa) | |||
GCLC | Glutamate-cysteine ligase catalytic subunit (637 aa) | |||
GFPT2 | Glutamine--fructose-6-phosphate aminotransferase [isomerizing] 2; Controls the flux of glucose into the hexosamine pathway. Most likely involved in regulating the availability of precursors for N- and O-linked glycosylation of proteins (682 aa) | |||
ASPA | Aspartoacylase; Catalyzes the deacetylation of N-acetylaspartic acid (NAA) to produce acetate and L-aspartate. NAA occurs in high concentration in brain and its hydrolysis NAA plays a significant part in the maintenance of intact white matter. In other tissues it act as a scavenger of NAA from body fluids (313 aa) | |||
PPAT | Amidophosphoribosyltransferase; Phosphoribosyl pyrophosphate amidotransferase; In the C-terminal section; belongs to the purine/pyrimidine phosphoribosyltransferase family (517 aa) | |||
CAD | CAD protein; This protein is a "fusion" protein encoding four enzymatic activities of the pyrimidine pathway (GATase, CPSase, ATCase and DHOase); In the central section; belongs to the metallo- dependent hydrolases superfamily. DHOase family. CAD subfamily (2225 aa) | |||
L2HGDH | L-2-hydroxyglutarate dehydrogenase, mitochondrial; L-2-hydroxyglutarate dehydrogenase; Belongs to the L2HGDH family (463 aa) | |||
TP53 | Cellular tumor antigen p53; Acts as a tumor suppressor in many tumor types; induces growth arrest or apoptosis depending on the physiological circumstances and cell type. Involved in cell cycle regulation as a trans-activator that acts to negatively regulate cell division by controlling a set of genes required for this process. One of the activated genes is an inhibitor of cyclin-dependent kinases. Apoptosis induction seems to be mediated either by stimulation of BAX and FAS antigen expression, or by repression of Bcl-2 expression. In cooperation with mitochondrial PPIF is involved in [...] (393 aa) | |||
AMT | Aminomethyltransferase, mitochondrial; The glycine cleavage system catalyzes the degradation of glycine (403 aa) | |||
GLUD1 | Glutamate dehydrogenase 1, mitochondrial; Mitochondrial glutamate dehydrogenase that converts L- glutamate into alpha-ketoglutarate. Plays a key role in glutamine anaplerosis by producing alpha-ketoglutarate, an important intermediate in the tricarboxylic acid cycle. May be involved in learning and memory reactions by increasing the turnover of the excitatory neurotransmitter glutamate (By similarity); Belongs to the Glu/Leu/Phe/Val dehydrogenases family (558 aa) | |||
FTCD | Formimidoyltransferase-cyclodeaminase; Folate-dependent enzyme, that displays both transferase and deaminase activity. Serves to channel one-carbon units from formiminoglutamate to the folate pool; In the N-terminal section; belongs to the formiminotransferase family (541 aa) | |||
GLUL | Glutamine synthetase; This enzyme has 2 functions- it catalyzes the production of glutamine and 4-aminobutanoate (gamma-aminobutyric acid, GABA), the latter in a pyridoxal phosphate-independent manner (By similarity). Essential for proliferation of fetal skin fibroblasts; Belongs to the glutamine synthetase family (373 aa) | |||
GLS2 | Glutaminase liver isoform, mitochondrial; Plays an important role in the regulation of glutamine catabolism. Promotes mitochondrial respiration and increases ATP generation in cells by catalyzing the synthesis of glutamate and alpha-ketoglutarate. Increases cellular anti-oxidant function via NADH and glutathione production. May play a role in preventing tumor proliferation; Ankyrin repeat domain containing (602 aa) | |||
GLS | Glutaminase kidney isoform, mitochondrial; Catalyzes the first reaction in the primary pathway for the renal catabolism of glutamine. Plays a role in maintaining acid-base homeostasis. Regulates the levels of the neurotransmitter glutamate in the brain. Isoform 2 lacks catalytic activity; Ankyrin repeat domain containing (669 aa) | |||
GCSH | Glycine cleavage system H protein, mitochondrial; The glycine cleavage system catalyzes the degradation of glycine. The H protein (GCSH) shuttles the methylamine group of glycine from the P protein (GLDC) to the T protein (GCST); Belongs to the GcvH family (173 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) | |||
GLUD2 | Glutamate dehydrogenase 2, mitochondrial; Important for recycling the chief excitatory neurotransmitter, glutamate, during neurotransmission; Belongs to the Glu/Leu/Phe/Val dehydrogenases family (558 aa) | |||
GGT7 | Glutathione hydrolase 7; Cleaves glutathione conjugates; Gamma-glutamyltransferases (662 aa) | |||
GFPT1 | Glutamine--fructose-6-phosphate aminotransferase [isomerizing] 1; Controls the flux of glucose into the hexosamine pathway. Most likely involved in regulating the availability of precursors for N- and O-linked glycosylation of proteins. Regulates the circadian expression of clock genes ARNTL/BMAL1 and CRY1 (699 aa) | |||
GAD1 | Glutamate decarboxylase 1; Catalyzes the production of GABA; Belongs to the group II decarboxylase family (594 aa) | |||
CNDP1 | Carnosine dipeptidase 1; Belongs to the peptidase M20A family (507 aa) | |||
EPRS | Bifunctional glutamate/proline--tRNA ligase; Catalyzes the attachment of the cognate amino acid to the corresponding tRNA in a two-step reaction- the amino acid is first activated by ATP to form a covalent intermediate with AMP and is then transferred to the acceptor end of the cognate tRNA. Component of the GAIT (gamma interferon-activated inhibitor of translation) complex which mediates interferon-gamma-induced transcript- selective translation inhibition in inflammation processes. Upon interferon-gamma activation and subsequent phosphorylation dissociates from the multisynthetase co [...] (1512 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) | |||
GAD2 | Glutamate decarboxylase 2; Catalyzes the production of GABA (585 aa) | |||
CPS1 | Carbamoyl-phosphate synthase [ammonia], mitochondrial; Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell (1506 aa) | |||
C14orf159 | D-glutamate cyclase, mitochondrial; D-glutamate cyclase that converts D-glutamate to 5-oxo- D-proline (621 aa) |