Showing metabocard for Oxoglutaric acid (BMDB0000208)

General function:

Amino acid transport and metabolism

Specific function:

Not Available

Gene Name:

BCAT1

Uniprot ID:

A4IFQ7

Molecular weight:

43185.0

General function:

Amino acid transport and metabolism

Specific function:

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 (By similarity).

Gene Name:

BCAT2

Uniprot ID:

Q5EA40

Molecular weight:

44648.0

Reactions

General function:

Amino acid transport and metabolism

Specific function:

Not Available

Gene Name:

BCAT2

Uniprot ID:

Q0V8J6

Molecular weight:

44274.0

General function:

Amino acid transport and metabolism

Specific function:

Not Available

Gene Name:

BCAT2

Uniprot ID:

Q5E9U7

Molecular weight:

40447.0

General function:

Transcription

Specific function:

Transaminase with broad substrate specificity. Has transaminase activity towards aminoadipate, kynurenine, methionine and glutamate. Shows activity also towards tryptophan, aspartate and hydroxykynurenine. Accepts a variety of oxo-acids as amino-group acceptors, with a preference for 2-oxoglutarate, 2-oxocaproic acid, phenylpyruvate and alpha-oxo-gamma-methiol butyric acid. Can also use glyoxylate as amino-group acceptor (in vitro) (By similarity).

Gene Name:

AADAT

Uniprot ID:

Q5E9N4

Molecular weight:

47901.0

Reactions

General function:

Involved in 2-hydroxyglutarate dehydrogenase activity

Specific function:

Not Available

Gene Name:

L2HGDH

Uniprot ID:

A7MBI3

Molecular weight:

51022.0

General function:

Energy production and conversion

Specific function:

Plays a role in intermediary metabolism and energy production. It may tightly associate or interact with the pyruvate dehydrogenase complex.

Gene Name:

IDH2

Uniprot ID:

Q04467

Molecular weight:

50739.0

General function:

Energy production and conversion

Specific function:

Regulatory subunit which plays a role in the allosteric regulation of the enzyme catalyzing the decarboxylation of isocitrate (ICT) into alpha-ketoglutarate. The heterodimer composed of the alpha (IDH3A) and beta (IDH3B) subunits and the heterodimer composed of the alpha (IDH3A) and gamma (IDH3G) subunits, have considerable basal activity but the full activity of the heterotetramer (containing two subunits of IDH3A, one of IDH3B and one of IDH3G) requires the assembly and cooperative function of both heterodimers.

Gene Name:

IDH3G

Uniprot ID:

Q58CP0

Molecular weight:

42863.0

General function:

Energy production and conversion

Specific function:

Plays a structural role to facilitate the assembly and ensure the full activity of the enzyme catalyzing the decarboxylation of isocitrate (ICT) into alpha-ketoglutarate. The heterodimer composed of the alpha (IDH3A) and beta (IDH3B) subunits and the heterodimer composed of the alpha (IDH3A) and gamma (IDH3G) subunits, have considerable basal activity but the full activity of the heterotetramer (containing two subunits of IDH3A, one of IDH3B and one of IDH3G) requires the assembly and cooperative function of both heterodimers.

Gene Name:

IDH3B

Uniprot ID:

O77784

Molecular weight:

42497.0

General function:

Energy production and conversion

Specific function:

May act as a corneal epithelial crystallin and may be involved in maintaining corneal epithelial transparency.

Gene Name:

IDH1

Uniprot ID:

Q9XSG3

Molecular weight:

46785.0

Reactions

General function:

Energy production and conversion

Specific function:

Catalytic subunit of the enzyme which catalyzes the decarboxylation of isocitrate (ICT) into alpha-ketoglutarate. The heterodimer composed of the alpha (IDH3A) and beta (IDH3B) subunits and the heterodimer composed of the alpha (IDH3A) and gamma (IDH3G) subunits, have considerable basal activity but the full activity of the heterotetramer (containing two subunits of IDH3A, one of IDH3B and one of IDH3G) requires the assembly and cooperative function of both heterodimers.

Gene Name:

IDH3A

Uniprot ID:

P41563

Molecular weight:

39668.0

General function:

Amino acid transport and metabolism

Specific function:

Biosynthesis of L-glutamate from L-aspartate or L-cysteine. Important regulator of levels of glutamate, the major excitatory neurotransmitter of the vertebrate central nervous system. Acts as a scavenger of glutamate in brain neuroprotection. The aspartate aminotransferase activity is involved in hepatic glucose synthesis during development and in adipocyte glyceroneogenesis. Using L-cysteine as substrate, regulates levels of mercaptopyruvate, an important source of hydrogen sulfide. Mercaptopyruvate is converted into H(2)S via the action of 3-mercaptopyruvate sulfurtransferase (3MST). Hydrogen sulfide is an important synaptic modulator and neuroprotectant in the brain (By similarity).

Gene Name:

GOT1

Uniprot ID:

P33097

Molecular weight:

46399.0

Reactions

General function:

Posttranslational modification, protein turnover, chaperones

Specific function:

Specifically hydroxylates an Asp or Asn residue in certain epidermal growth factor-like (EGF) domains of a number of proteins.

Gene Name:

ASPH

Uniprot ID:

Q28056

Molecular weight:

84999.0

General function:

Amino acid transport and metabolism

Specific function:

Catalyzes the irreversible transamination of the L-tryptophan metabolite L-kynurenine to form kynurenic acid (KA). Plays a key role in amino acid metabolism. Important for metabolite exchange between mitochondria and cytosol. Facilitates cellular uptake of long-chain free fatty acids (By similarity).

Gene Name:

GOT2

Uniprot ID:

P12344

Molecular weight:

47514.0

Reactions

General function:

Amino acid transport and metabolism

Specific function:

Not Available

Gene Name:

GOT1L1

Uniprot ID:

Q2T9S8

Molecular weight:

45918.0

General function:

Energy production and conversion

Specific function:

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 (By similarity).

Gene Name:

ADHFE1

Uniprot ID:

A6QP15

Molecular weight:

50343.0

General function:

Amino acid transport and metabolism

Specific function:

Catalyzes the conversion of gamma-aminobutyrate and L-beta-aminoisobutyrate to succinate semialdehyde and methylmalonate semialdehyde, respectively. Can also convert delta-aminovalerate and beta-alanine (By similarity).

Gene Name:

ABAT

Uniprot ID:

Q9BGI0

Molecular weight:

56731.0

Reactions

General function:

Secondary metabolites biosynthesis, transport and catabolism

Specific function:

Converts trimethyllysine (TML) into hydroxytrimethyllysine (HTML).

Gene Name:

TMLHE

Uniprot ID:

Q0VC74

Molecular weight:

49837.0

Reactions

General function:

Amino acid transport and metabolism

Specific function:

Bifunctional enzyme that catalyzes the first two steps in lysine degradation. The N-terminal and the C-terminal contain lysine-oxoglutarate reductase and saccharopine dehydrogenase activity, respectively (By similarity).

Gene Name:

AASS

Uniprot ID:

A8E657

Molecular weight:

102084.0

Reactions

General function:

Involved in iron ion binding

Specific function:

Catalyzes the post-translational formation of 4-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.

Gene Name:

P4HA1

Uniprot ID:

Q1RMU3

Molecular weight:

61010.0

General function:

Involved in iron ion binding

Specific function:

Part of a complex composed of PLOD1, P3H3 and P3H4 that catalyzes hydroxylation of lysine residues in collagen alpha chains and is required for normal assembly and cross-linkling of collagen fibrils (By similarity). Forms hydroxylysine residues in -Xaa-Lys-Gly- sequences in collagens (By similarity). These hydroxylysines serve as sites of attachment for carbohydrate units and are essential for the stability of the intermolecular collagen cross-links (By similarity).

Gene Name:

PLOD1

Uniprot ID:

O77588

Molecular weight:

83487.0

General function:

Involved in iron ion binding

Specific function:

Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.

Gene Name:

PHYH

Uniprot ID:

O18778

Molecular weight:

38770.0

Reactions

General function:

Involved in iron ion binding

Specific function:

Catalyzes the post-translational formation of 4-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.

Gene Name:

P4HA3

Uniprot ID:

Q75UG4

Molecular weight:

61023.0

Reactions

General function:

Energy production and conversion

Specific function:

Catalyzes the oxidation of D-2-hydroxyglutarate to alpha-ketoglutarate.

Gene Name:

D2HGDH

Uniprot ID:

Q1JPD3

Molecular weight:

59056.0

General function:

Coenzyme transport and metabolism

Specific function:

Not Available

Gene Name:

PSAT1

Uniprot ID:

A6QR28

Molecular weight:

40529.0

Reactions

General function:

Replication, recombination and repair

Specific function:

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.

Gene Name:

GLUD1

Uniprot ID:

P00366

Molecular weight:

61512.0

Reactions

General function:

Amino acid transport and metabolism

Specific function:

Catalyzes the reversible transamination between alanine and 2-oxoglutarate to form pyruvate and glutamate. Participates in cellular nitrogen metabolism and also in liver gluconeogenesis starting with precursors transported from skeletal muscles (By similarity).

Gene Name:

GPT

Uniprot ID:

A4IFH5

Molecular weight:

55275.0

General function:

Amino acid transport and metabolism

Specific function:

Transaminase involved in tyrosine breakdown. Converts tyrosine to p-hydroxyphenylpyruvate. Can catalyze the reverse reaction, using glutamic acid, with 2-oxoglutarate as cosubstrate (in vitro). Has much lower affinity and transaminase activity for phenylalanine (By similarity).

Gene Name:

TAT

Uniprot ID:

Q58CZ9

Molecular weight:

49691.0

Reactions

General function:

Amino acid transport and metabolism

Specific function:

Catalyzes the irreversible transamination of the L-tryptophan metabolite L-kynurenine to form kynurenic acid (KA). May catalyze the beta-elimination of S-conjugates and Se-conjugates of L-(seleno)cysteine, resulting in the cleavage of the C-S or C-Se bond (By similarity). Has transaminase activity towards L-kynurenine, tryptophan, phenylalanine, serine, cysteine, methionine, histidine, glutamine and asparagine with glyoxylate as an amino group acceptor (in vitro). Has lower activity with 2-oxoglutarate as amino group acceptor (in vitro) (By similarity).

Gene Name:

KYAT3

Uniprot ID:

Q0P5G4

Molecular weight:

51472.0

General function:

Coenzyme transport and metabolism

Specific function:

Catalyzes the reversible oxidation of 3-phospho-D-glycerate to 3-phosphonooxypyruvate, the first step of the phosphorylated L-serine biosynthesis pathway. Also catalyzes the reversible oxidation of 2-hydroxyglutarate to 2-oxoglutarate and the reversible oxidation of (S)-malate to oxaloacetate.

Gene Name:

PHGDH

Uniprot ID:

Q5EAD2

Molecular weight:

56452.0

General function:

Energy production and conversion

Specific function:

2-oxoglutarate dehydrogenase (E1) component of the 2-oxoglutarate dehydrogenase complex, which mediates the decarboxylation of alpha-ketoglutarate. The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). The 2-oxoglutarate dehydrogenase complex is mainly active in the mitochondrion. A fraction of the 2-oxoglutarate dehydrogenase complex also localizes in the nucleus and is required for lysine succinylation of histones: associates with KAT2A on chromatin and provides succinyl-CoA to histone succinyltransferase KAT2A.

Gene Name:

OGDH

Uniprot ID:

Q148N0

Molecular weight:

115808.0

General function:

Amino acid transport and metabolism

Specific function:

Not Available

Gene Name:

OAT

Uniprot ID:

Q3ZCF5

Molecular weight:

48075.0

Reactions

General function:

Involved in binding

Specific function:

Catalyzes the transport of 2-oxoglutarate across the inner mitochondrial membrane in an electroneutral exchange for malate or other dicarboxylic acids, and plays an important role in several metabolic processes, including the malate-aspartate shuttle, the oxoglutarate/isocitrate shuttle, in gluconeogenesis from lactate, and in nitrogen metabolism. Maintains mitochondrial fusion and fission events, and the organization and morphology of cristae. Involved in the regulation of apoptosis.

Gene Name:

SLC25A11

Uniprot ID:

P22292

Molecular weight:

34172.0

General function:

Involved in binding

Specific function:

Not Available

Gene Name:

SLC25A11

Uniprot ID:

A5D954

Molecular weight:

33899.0

General function:

Not Available

Specific function:

Not Available

Gene Name:

EARS2

Uniprot ID:

G3N260

Molecular weight:

58494.0

Reactions