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SUCLG1 encodes the alpha subunit of the heterodimeric enzyme succinate coenzyme A ligase. Additionally we are shipping Succinate-CoA Ligase, alpha Subunit Proteins (7) and many more products for this protein.
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Long survival, to age 20 years or older, was reported in 12% of SUCLA2 (show SUCLA2 Antibodies) and in 10% of SUCLG1 patients.
First Chinese report of succinyl-CoA ligase (show SUCLG2 Antibodies) deficiency caused by novel SUCLG1 mutations; five novel pathogenic mutations in SUCLG1 were identified
3 novel mutations have been identified in patients with the mitochondrial DNA depletion syndrome (c.1048G>A and c.1049G>T in SUCLA2 (show SUCLA2 Antibodies) and c.531+4A>T in SUCLG1).
Our results suggest that SUCLG1 mutations that lead to complete absence of SUCLG1 protein are responsible for a very severe disorder with antenatal manifestations, whereas a SUCLA2 (show SUCLA2 Antibodies)-like phenotype is found in patients with residual SUCLG1 protein.
This report enlarges the phenotypic spectrum of SUCLG1 mutations and confirms that a characteristic metabolic profile (presence of MMA and C4-DC carnitine in urines) and basal ganglia MRI (show C7ORF49 Antibodies) lesions are the hallmarks of SCS (show TWIST1 Antibodies) defects.
A novel mutation in the SUCLG1 gene was found in two newborns having lethal lactic acidosis, multi-organ failure and congenital malformations including interrupted aortic arch.
Structure of GTP-specific succinyl-CoA synthetase subunits in complex with CoA has been resolved.
succinyl-CoA (show OXCT1 Antibodies) synthetase is able to protect GTP (show AK3 Antibodies) from hydrolysis when the active-site histidine residue is not in position to be phosphorylated
ADP and GDP succinyl-CoA (show OXCT1 Antibodies) synthetases have different expression patterns and specificity
This gene encodes the alpha subunit of the heterodimeric enzyme succinate coenzyme A ligase. This enzyme is targeted to the mitochondria and catalyzes the conversion of succinyl CoA and ADP or GDP to succinate and ATP or GTP. Mutations in this gene are the cause of the metabolic disorder fatal infantile lactic acidosis and mitochondrial DNA depletion.
succinate-CoA ligase, GDP-forming, alpha subunit
, succinate-CoA ligase, GDP-forming alpha subunit
, succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial
, succinyl-CoA ligase [GDP-forming] subunit alpha, mitochondrial
, succinyl-CoA synthetase subunit alpha
, succinyl-CoA synthetase alpha subunit