NP_003841
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NCBI GenBank Nucleotide #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
48,040 Da
NCBI Official Full Name
succinyl-CoA ligase
NCBI Official Synonym Full Names
succinate-CoA ligase, ADP-forming, beta subunit
NCBI Protein Information
succinyl-CoA ligase [ADP-forming] subunit beta, mitochondrial
UniProt Protein Name
Succinyl-CoA ligase [ADP-forming] subunit beta, mitochondrial
UniProt Synonym Protein Names
ATP-specific succinyl-CoA synthetase subunit beta; Renal carcinoma antigen NY-REN-39; Succinyl-CoA synthetase beta-A chain; SCS-betaA
UniProt Synonym Gene Names
UniProt Entry Name
SUCB1_HUMAN
NCBI Summary for SUCLA2
Succinyl-CoA synthetase (SCS) is a mitochondrial matrix enzyme that acts as a heterodimer, being composed of an invariant alpha subunit and a substrate-specific beta subunit. The protein encoded by this gene is an ATP-specific SCS beta subunit that dimerizes with the SCS alpha subunit to form SCS-A, an essential component of the tricarboxylic acid cycle. SCS-A hydrolyzes ATP to convert succinate to succinyl-CoA. Defects in this gene are a cause of myopathic mitochondrial DNA depletion syndrome. A pseudogene of this gene has been found on chromosome 6. [provided by RefSeq, Jul 2008]
UniProt Comments for SUCLA2
SCS-beta: a mitochondrial enzyme that catalyzes the phosphorylation of NTP, where N = adenosine or guanosine, in the tricarboxylic acid cycle. Catalyzes the following: ATP + succinate + CoA = ADP + succinyl-CoA + phosphate. Two splice variant isoforms have been described.
Protein type: Ligase; Carbohydrate Metabolism - propanoate; EC 6.2.1.5; Mitochondrial; Carbohydrate Metabolism - citrate (TCA) cycle
Chromosomal Location of Human Ortholog: 13q14.2
Cellular Component: mitochondrion; mitochondrial matrix
Molecular Function: protein binding; metal ion binding; succinate-CoA ligase (ADP-forming) activity; ATP binding
Biological Process: succinate metabolic process; cellular metabolic process; succinyl-CoA pathway; tricarboxylic acid cycle; succinyl-CoA metabolic process
Disease: Mitochondrial Dna Depletion Syndrome 5 (encephalomyopathic With Or Without Methylmalonic Aciduria)
Research Articles on SUCLA2
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Pathways associated with anti-SUCLA2 antibody
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Pathway Diagram |
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Biosynthesis Of Antibiotics Pathway Diagram |
Carbon Metabolism Pathway antibodies |
Carbon Metabolism Pathway Diagram |
Carbon Metabolism Pathway antibodies |
Carbon Metabolism Pathway Diagram |
Citrate Cycle (TCA Cycle) Pathway antibodies |
Citrate Cycle (TCA Cycle) Pathway Diagram |
Citrate Cycle (TCA Cycle) Pathway antibodies |
Citrate Cycle (TCA Cycle) Pathway Diagram |
Citrate Cycle (TCA Cycle, Krebs Cycle) Pathway antibodies |
Citrate Cycle (TCA Cycle, Krebs Cycle) Pathway Diagram |
Citrate Cycle (TCA Cycle, Krebs Cycle) Pathway antibodies |
Citrate Cycle (TCA Cycle, Krebs Cycle) Pathway Diagram |
Citrate Cycle, Second Carbon Oxidation, 2-oxoglutarate => Oxaloacetate Pathway antibodies |
Citrate Cycle, Second Carbon Oxidation, 2-oxoglutarate => Oxaloacetate Pathway Diagram |
Citrate Cycle, Second Carbon Oxidation, 2-oxoglutarate => Oxaloacetate Pathway antibodies |
Citrate Cycle, Second Carbon Oxidation, 2-oxoglutarate => Oxaloacetate Pathway Diagram |
Citric Acid Cycle (TCA Cycle) Pathway antibodies |
Citric Acid Cycle (TCA Cycle) Pathway Diagram |
Diseases associated with anti-SUCLA2 antibody
Organs/Tissues associated with anti-SUCLA2 antibody
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