Showing metabocard for Propionyl-CoA (BMDB0001275)

General function:

Involved in ATP binding

Specific function:

This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites. Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA (By similarity). Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain, while the beta subunit then transfers the carboxyl group from carboxylated biotin to propionyl-CoA (By similarity). Propionyl-CoA carboxylase also significantly acts on butyryl-CoA/butanoyl-CoA, which is converted to ethylmalonyl-CoA/(2S)-ethylmalonyl-CoA (By similarity). Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA (By similarity).

Gene Name:

PCCB

Uniprot ID:

Q2TBR0

Molecular weight:

58311.0

Reactions

General function:

Energy production and conversion

Specific function:

Plays a role in valine and pyrimidine metabolism. Binds fatty acyl-CoA.

Gene Name:

ALDH6A1

Uniprot ID:

Q07536

Molecular weight:

58063.0

Reactions

General function:

Lipid transport and metabolism

Specific function:

Catalyzes the synthesis of acetyl-CoA from short-chain fatty acids (By similarity). Propionate is the preferred substrate but can also utilize acetate and butyrate with a much lower affinity.

Gene Name:

ACSS3

Uniprot ID:

A7MB45

Molecular weight:

74805.0

Reactions

General function:

Lipid transport and metabolism

Specific function:

In the production of energy from fats, this is one of the enzymes that catalyzes the last step of the mitochondrial beta-oxidation pathway, an aerobic process breaking down fatty acids into acetyl-CoA. Using free coenzyme A/CoA, catalyzes the thiolytic cleavage of medium- to long-chain unbranched 3-oxoacyl-CoAs into acetyl-CoA and a fatty acyl-CoA shortened by two carbon atoms. Also catalyzes the condensation of two acetyl-CoA molecules into acetoacetyl-CoA and could be involved in the production of ketone bodies. Also displays hydrolase activity on various fatty acyl-CoAs (By similarity). Thereby, could be responsible for the production of acetate in a side reaction to beta-oxidation (By similarity). Abolishes BNIP3-mediated apoptosis and mitochondrial damage (By similarity).

Gene Name:

ACAA2

Uniprot ID:

Q3T0R7

Molecular weight:

42131.0

Reactions

General function:

Lipid transport and metabolism

Specific function:

Mediates in vitro the transfer of all common phospholipids, cholesterol and gangliosides between membranes. May play a role in regulating steroidogenesis.

Gene Name:

SCP2

Uniprot ID:

P07857

Molecular weight:

58577.0

General function:

Involved in ATP binding

Specific function:

Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin in the first step and the transfer of the carboxyl group to pyruvate in the second. Catalyzes in a tissue specific manner, the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis from pyruvate (By similarity).

Gene Name:

PC

Uniprot ID:

Q29RK2

Molecular weight:

129698.0

Reactions

General function:

Energy production and conversion

Specific function:

The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).

Gene Name:

BCKDHA

Uniprot ID:

P11178

Molecular weight:

51678.0

Reactions

General function:

Energy production and conversion

Specific function:

The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).

Gene Name:

BCKDHB

Uniprot ID:

P21839

Molecular weight:

42935.0

Reactions

General function:

Energy production and conversion

Specific function:

The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). Within this complex, the catalytic function of this enzyme is to accept, and to transfer to coenzyme A, acyl groups that are generated by the branched-chain alpha-keto acid decarboxylase component.

Gene Name:

DBT

Uniprot ID:

P11181

Molecular weight:

53410.0

Reactions

General function:

Lipid transport and metabolism

Specific function:

Acyl-CoA dehydrogenase specific for acyl chain lengths of 4 to 16 that catalyzes the initial step of fatty acid beta-oxidation. Utilizes the electron transfer flavoprotein (ETF) as an electron acceptor to transfer electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase).

Gene Name:

ACADM

Uniprot ID:

Q3SZB4

Molecular weight:

46573.0

Reactions

General function:

Not Available

Specific function:

Not Available

Gene Name:

DLD

Uniprot ID:

F1N206

Molecular weight:

54187.0

Reactions

General function:

Not Available

Specific function:

Not Available

Gene Name:

AceCS2

Uniprot ID:

Q9BEA3

Molecular weight:

74310.0

Reactions