Succinate--CoA ligase [GDP-forming] subunit beta, mitochondrial

SUCLG2 encodes the beta subunit of succinate–CoA ligase [GDP-forming], a mitochondrial enzyme that catalyzes GTP-specific succinyl-CoA hydrolysis during the citric acid cycle (UniProt: Q96I99). This subunit provides nucleotide specificity and binds substrate succinate, enabling substrate-level phosphorylation and contributing to cellular energy metabolism. The enzyme also demonstrates activity toward itaconyl- and malyl-CoA.

SUCLG2 is expressed in mitochondrial tissue and functions within the tricarboxylic acid cycle pathway. No baseline disease associations are documented in UniProt; however, the protein has been identified as relevant to neurodegeneration.

In Alzheimer's disease, SUCLG2 is upregulated in post-mortem AD brain tissue relative to age-matched controls, with a mean log2 fold-change of +0.69 (Chaparral AD proteomics). This increase was detected across subcellular fractions in a TMT-labeled proteomics study of human post-mortem brain samples, suggesting enhanced mitochondrial energy metabolism or compensatory metabolic activity in AD pathology.