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]
| SKU | Test Kits | Buffer | Dye Color | Order Now |
|---|---|---|---|---|
| SUCLA2-20-RE | 20 (40 μL) | 200 μL |  |
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| SUCLA2-20-OR | 20 (40 μL) | 200 μL |  |
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| SUCLA2-20-GO | 20 (40 μL) | 200 μL |  |
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| SUCLA2-20-GR | 20 (40 μL) | 200 μL |  |
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| SUCLA2-20-AQ | 20 (40 μL) | 200 μL |  |
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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]
Gene Symbol : SUCLA2
Gene Name : Succinate-CoA ligase ADP-forming beta subunit
Chromosome : CHR 13: 480,379,71-479,426,55
Locus : 13q14.2
Alt. Genes : ZNF263
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