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SUCLA2 gene

Known as: A-BETA, SUCCINATE-CoA LIGASE, ADP-FORMING, BETA SUBUNIT, ATP-SPECIFIC SUCCINYL-CoA SYNTHETASE, BETA SUBUNIT 
National Institutes of Health

Papers overview

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Review
2016
Review
2016
Succinate-CoA ligase deficiency due to mutations in SUCLA2 and SUCLG1: phenotype and genotype correlations in 71 patients
BackgroundThe encephalomyopathic mtDNA depletion syndrome with methylmalonic aciduria is associated with deficiency of succinate… 
2016
2016
Two transgenic mouse models for β-subunit components of succinate-CoA ligase yielding pleiotropic metabolic alterations
Succinate-CoA ligase (SUCL) is a heterodimer enzyme composed of Suclg1 α-subunit and a substrate-specific Sucla2 or Suclg2… 
2016
2016
Knockdown of Sucla2 decreases the viability of mouse spermatocytes by inducing apoptosis through injury of the mitochondrial function of cells.
INTRODUCTION Sucla2, a β subunit of succinyl coenzyme A synthase, is located in the mitochondrial matrix. Sucla2 catalyzes the… 
Review
2014
Review
2014
Mitochondrial encephalomyopathy and retinoblastoma explained by compound heterozygosity of SUCLA2 point mutation and 13q14 deletion
Mutations in SUCLA2, encoding the ß-subunit of succinyl-CoA synthetase of Krebs cycle, are one cause of mitochondrial DNA… 
Highly Cited
2011
Highly Cited
2011
The interplay between SUCLA2, SUCLG2, and mitochondrial DNA depletion.
2009
2009
Dystonia and deafness due to SUCLA2 defect; Clinical course and biochemical markers in 16 children.
Review
2008
Review
2008
Inherited mitochondrial diseases of DNA replication.
Mitochondrial genetic diseases can result from defects in mitochondrial DNA (mtDNA) in the form of deletions, point mutations, or… 
Highly Cited
2007
Highly Cited
2007
SUCLA2 mutations are associated with mild methylmalonic aciduria, Leigh-like encephalomyopathy, dystonia and deafness.
One pedigree with four patients has been recently described with mitochondrial DNA depletion and mutation in SUCLA2 gene leading… 
Highly Cited
2007
Highly Cited
2007
Mitochondrial encephalomyopathy with elevated methylmalonic acid is caused by SUCLA2 mutations.
We have identified 12 patients with autosomal recessive mitochondrial encephalomyopathy with elevated methylmalonic acid. The… 
Highly Cited
2006
Highly Cited
2006
MPV17 encodes an inner mitochondrial membrane protein and is mutated in infantile hepatic mitochondrial DNA depletion
The mitochondrial (mt) DNA depletion syndromes (MDDS) are genetic disorders characterized by a severe, tissue-specific decrease… 
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