SUCLG1 Gene Summary [Human]
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. [provided by RefSeq, Feb 2010]
Details
| Type | Protein Coding |
| Official Symbol | SUCLG1 |
| Official Name | succinate-CoA ligase GDP/ADP-forming subunit alpha [Source:HGNC Symbol;Acc:HGNC:11449] |
| Ensembl ID | ENSG00000163541 |
| Bio databases IDs | |
| Aliases | succinate-CoA ligase GDP/ADP-forming subunit alpha |
| Synonyms | 1500000I01Rik, GALPHA, MTDPS9, SCS-alpha, SCS-α, Succinate-CoA ligase, succinate-CoA ligase GDP/ADP-forming subunit alpha, succinate-CoA ligase GDP/ADP-forming subunit α, succinate-CoA ligase, GDP-forming, alpha subunit, succinate-CoA ligase, GDP-forming, α subunit, Succinyl-CoA Synthetase Alpha, Succinyl-CoA Synthetase α, SUCLA1 |
| Species | Human, Homo sapiens |
| Orthologies |
Protein Domains
A protein domain is a distinct structural or functional region within a protein that can evolve, function, and exist independently of the rest of the protein chain. These domains in human SUCLG1 often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- CoA binding domain
- Rossmann-fold NAD(P)(+)-binding proteins
- succinyl-CoA synthetase, alpha subunit
- binding protein
- succinate-CoA ligase (GDP-forming)
- GDP binding
- succinate-CoA ligase (ADP-forming)
- CoA-ligase
- enzyme
- protein binding
Top Findings
The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
- insulin resistance
- Alzheimer disease
- mitochondrial DNA depletion syndrome 9 with methylmalonic aciduria
- idiopathic dilated cardiomyopathy
- Leigh syndrome
role in cell
- proliferation
- expression in
- accumulation in
- number
- succinylation in
Subcellular Expression
Locations within the cell where the protein is known or predicted to be active, providing insight into its function and cellular context.
- Cytoplasm
- cellular membrane
- Nucleus
- Mitochondria
- cytosol
- mitochondrial matrix
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the human SUCLG1 gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- tricarboxylic acid cycle
Cellular Component
Where in the cell the gene product is active
- succinate-CoA ligase complex (ADP-forming)
- mitochondrial matrix
- succinate-CoA ligase complex (GDP-forming)
- mitochondrion
Molecular Function
What the gene product does at the molecular level
- nucleotide binding
- succinate-CoA ligase (GDP-forming) activity
- protein binding
- RNA binding
- succinate-CoA ligase (ADP-forming) activity