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Article

Disorders caused by deficiency of succinate-CoA ligase.

Journal of inherited metabolic disease - 1 Apr 2008

Ostergaard E

Abstract excerpt

Succinate-CoA ligase catalyses the reversible conversion of succinyl-CoA and ADP or GDP to succinate and ATP or GTP. It is a mitochondrial matrix enzyme and at least the ADP-forming enzyme is part of the Krebs cycle. The substrate specificity is determined by the beta subunit of succinate-CoA ligase, which is encoded by either SUCLA2 or SUCLG2. In patients with severe hypotonia, deafness and Leigh-like syndrome,...

Read the complete abstract on PubMed

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Share to Citric Acid Cycle
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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

Identifiers

PMID: 18392745
DOI: 10.1007/s10545-008-0828-7

Genes

SUCLA2
SUCLG1
SUCLG2

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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Disorders caused by deficiency of succinate-CoA ligase.PMID 18392745Read ↗

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