Suclg1 Protein is involved in cellular processes relevant to neurodegeneration. For detailed information, refer to the References section.
SUCLG1 encodes the alpha subunit of succinyl-CoA synthetase (SCS), which catalyzes the reversible conversion of succinyl-CoA to succinate, coupled to GTP or ATP synthesis. [1]
| Protein Name | Succinyl-CoA Ligase GDP-Forming Subunit Alpha |
|---|---|
| Gene | [SUCLG1](/genes/suclg1) |
| UniProt ID | [P53567](https://www.uniprot.org/uniprot/P53567) |
| PDB Structure IDs | 1ZMS |
| Molecular Weight | 35 kDa |
| Subcellular Localization | Mitochondria (matrix) |
| Protein Family | Succinyl-CoA synthetase family |
Succinyl-CoA synthetase is a heterodimer of alpha (SUCLG1) and beta (SUCLG2 or SUCLA2) subunits. The alpha subunit contains the binding site for CoA and nucleoside diphosphates.
Succinyl-CoA synthetase (SCS) catalyzes the final step of the TCA cycle:
succinyl-CoA + GDP/ADP + Pi ↔ succinate + GTP/ATP + CoA
SCS is unique among TCA cycle enzymes because it directly produces nucleoside triphosphates rather than NADH. There are two isoforms:
The GTP-producing isoform predominates in most tissues, while the ATP-producing isoform is important in tissues with high energy demands.
In neurons, SCS supports energy production and maintains nucleotide pools for RNA and DNA synthesis.
Alzheimer Disease: SCS activity may be reduced in AD brains. Impaired GTP production may affect neuronal signaling and protein synthesis.
Mitochondrial DNA Depletion Syndrome: SUCLG1 mutations cause severe mitochondrial DNA depletion syndrome (MTDPS) with encephalomyopathy, characterized by profound muscle weakness and early death.
Cardiomyopathy: SUCLG1 mutations can cause cardiomyopathy as part of mitochondrial disease. The heart has extremely high energy demands.
Metabolic Syndrome: Altered SCS function may contribute to metabolic dysfunction in aging.
No direct SUCLG1-targeted drugs exist. Research focuses on: