Showing metabocard for Succinic acid (BMDB0000254)

General function:

Energy production and conversion

Specific function:

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).

Gene Name:

SDHB

Uniprot ID:

Q3T189

Molecular weight:

31518.0

General function:

Energy production and conversion

Specific function:

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) (Probable). Can act as a tumor suppressor (By similarity).

Gene Name:

SDHA

Uniprot ID:

P31039

Molecular weight:

72944.0

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:

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:

Energy production and conversion

Specific function:

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.

Gene Name:

SUCLA2

Uniprot ID:

Q148D5

Molecular weight:

50130.0

General function:

Energy production and conversion

Specific function:

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.

Gene Name:

SUCLG1

Uniprot ID:

Q58DR8

Molecular weight:

36167.0

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General function:

Energy production and conversion

Specific function:

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.

Gene Name:

SUCLG2

Uniprot ID:

Q3MHX5

Molecular weight:

46691.0

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:

Not Available

Specific function:

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.

Gene Name:

OXCT1

Uniprot ID:

Q24JZ7

Molecular weight:

56453.0

Reactions