SUCLG2


Description

The SUCLG2 (succinate-CoA ligase GDP-forming subunit beta) is a protein-coding gene located on chromosome 3.

Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrial is an enzyme encoded by the SUCLG2 gene on chromosome 3. This gene encodes a GTP-specific beta subunit of succinyl-CoA synthetase. Succinyl-CoA synthetase catalyzes the reversible reaction involving the formation of succinyl-CoA and succinate. Alternate splicing results in multiple transcript variants. Pseudogenes of this gene are found on chromosomes 5 and 12.

SCS, also known as succinyl CoA ligase (SUCL), is a heterodimer composed of a catalytic α subunit encoded by the SUCLG1 gene and a β subunit encoded by either the SUCLA2 gene or the SUCLG2 gene, which determines the enzyme specificity for either ADP or GDP. SUCLG2 is the SCS variant containing the SUCLG2-encoded β subunit. Amino acid sequence alignment of the two β subunit types reveals a homology of ~50% identity, with specific regions conserved throughout the sequences. SUCLG2 is located on chromosome 3 and contains 14 exons.

As a subunit of SCS, SUCLG2 is a mitochondrial matrix enzyme that catalyzes the reversible conversion of succinyl-CoA to succinate and acetoacetyl CoA, accompanied by the substrate-level phosphorylation of GDP to GTP, as a step in the tricarboxylic acid (TCA) cycle. The GTP generated is then consumed in anabolic pathways.

SUCLG2 is also known as G-SCS, GBETA, GTPSCS.

Associated Diseases


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