Acylcarnitines are fatty acid metabolites that play important roles in many cellular energy
metabolism pathways. They have historically been used as important diagnostic markers for …

Biallelic pathogenic variants in subunits of succinyl-CoA synthetase (SCS), a tricarboxylic
acid (TCA) cycle enzyme, are associated with mitochondrial encephalomyopathy in humans …

Elucidating the tumorigenic mechanism of R-2-hydroxyglutarate (R-2HG) is critical for
determining how NADP+-IDH mutations cause cancer. Here we report that R-2HG induces …

Background The encephalomyopathic mtDNA depletion syndrome with methylmalonic
aciduria is associated with deficiency of succinate-CoA ligase, caused by mutations in …

Lysine succinylation is a subtype of protein acylation associated with metabolic regulation of
succinyl-CoA in the tricarboxylic acid cycle. Deficiency of succinyl-CoA synthetase (SCS) …

Succinate-CoA ligase (SUCL) is a heterodimer enzyme composed of Suclg1 α-subunit and
a substrate-specific Sucla2 or Suclg2 β-subunit yielding ATP or GTP, respectively. In …

Protein O-GlcNAcylation is a dynamic posttranslational modification that is catalyzed by the
enzyme O-GlcNAc transferase (OGT) and is essential for neurodevelopment and postnatal …

Succinyl-CoA synthetase catalyzes the only step in the citric acid cycle that provides
substrate-level phosphorylation. Although the binding sites for the substrates CoA …

Mutations in SUCLA2 result in succinyl-CoA ligase (ATP-forming) or succinyl-CoA
synthetase (ADP-forming)(A-SCS) deficiency, a mitochondrial tricarboxylic acid cycle …

Rat hearts were perfused with [1, 2, 3, 4-13 C 4] palmitic acid (M+ 4), and the isotopic
patterns of myocardial acylcarnitines and acyl-CoAs were analyzed using ultra-HPLC …