SUCLA2 - succinate-CoA ligase ADP-forming subunit beta Gene
Also Known as A-SCS; A-BETA; MTDPS5; LINC00444; SCS-betaA
Species: Homo sapiens
About SUCLA2
This gene has 20 transcripts (splice variants), 230 orthologues, 1 paralogue and is associated with 2 phenotypes. Ubiquitous expression in heart (RPKM 24.1), fat (RPKM 23.0) and 25 other tissues.
Summary
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]
SUCLA2 Products (1)
| mRNA | Protein | Name |
|---|---|---|
| NM_003850.3 | NP_003841.1 | succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial precursor |
| Molecular Function GO Annotation | Evidence | Verweise | Source |
|---|---|---|---|
| enables protein binding |
IPI
IPI: Inferred from physical interaction
|
10727444 | GOA |
| Cellular Component GO Annotation | Evidence | Verweise | Source |
|---|---|---|---|
| located in mitochondrion |
IDA
IDA: Inferred from direct assay
|
10727444 | GOA |
SUCLA2 Protein Structure
ATP-grasp_2: ATP-grasp domain (55 - 262)
Ligase_CoA: CoA-ligase (321 - 441)
- 0
- 100
- 200
- 300
- 400
- 463 a.a.
| Protein Preferred Names | Protein Names | |
|---|---|---|
|
succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial |
|
SUCLA2 Protein-protein interaction Information
|
Type
|
Protein Name | Protein ID | Interactor | Interactor Species | Interactor ID | Detection Method | Verweise |
|---|---|---|---|---|---|---|---|
|
Intra
|
SUCLA2 | Q9P2R7 | TNFRSF10D | Homo sapiens | Q9UBN6 | 32296183 | |
|
Intra
|
SUCLA2 | Q9P2R7 | TNFRSF10D | Homo sapiens | Q9UBN6 | 32296183 | |
|
Intra
|
SUCLA2 | Q9P2R7 | TNFRSF10D | Homo sapiens | Q9UBN6 | 32296183 | |
|
Intra
|
SUCLA2 | Q9P2R7 | AGTRAP | Homo sapiens | Q6RW13-2 | 26871637 | |
|
Intra
|
SUCLA2 | Q9P2R7 | AGTRAP | Homo sapiens | Q6RW13-2 | 26871637 | |
|
Intra
|
SUCLA2 | Q9P2R7 | AGTRAP | Homo sapiens | Q6RW13-2 | 26871637 | |
|
Intra
|
SUCLA2 | Q9P2R7 | AGTRAP | Homo sapiens | Q6RW13-2 | 32296183 | |
|
Intra
|
SUCLA2 | Q9P2R7 | CMTM5 | Homo sapiens | Q96DZ9-2 | 26871637 | |
|
Intra
|
SUCLA2 | Q9P2R7 | CMTM5 | Homo sapiens | Q96DZ9-2 | 26871637 | |
|
Intra
|
SUCLA2 | Q9P2R7 | CMTM5 | Homo sapiens | Q96DZ9-2 | 32296183 | |
|
Intra
|
SUCLA2 | Q9P2R7 | CMTM5 | Homo sapiens | Q96DZ9-2 | 26871637 | |
|
Intra
|
SUCLA2 | Q9P2R7 | CMTM5 | Homo sapiens | Q96DZ9-2 | 32296183 | |
|
Intra
|
SUCLA2 | Q9P2R7 | FKBP7 | Homo sapiens | Q9Y680 | 32296183 | |
|
Intra
|
SUCLA2 | Q9P2R7 | FKBP7 | Homo sapiens | Q9Y680 | 32296183 | |
|
Intra
|
SUCLA2 | Q9P2R7 | FKBP7 | Homo sapiens | Q9Y680 | 32296183 | |
|
Intra
|
SUCLA2 | Q9P2R7 | ARL6IP1 | Homo sapiens | Q15041 | 26871637 | |
|
Intra
|
SUCLA2 | Q9P2R7 | ARL6IP1 | Homo sapiens | Q15041 | 32296183 | |
|
Intra
|
SUCLA2 | Q9P2R7 | ARL6IP1 | Homo sapiens | Q15041 | 26871637 | |
|
Intra
|
SUCLA2 | Q9P2R7 | ARL6IP1 | Homo sapiens | Q15041 | 32296183 | |
|
Intra
|
SUCLA2 | Q9P2R7 | ARL6IP1 | Homo sapiens | Q15041 | 26871637 | |
|
Intra
|
SUCLA2 | Q9P2R7 | MAGEA11 | Homo sapiens | P43364 | 32296183 | |
|
Intra
|
SUCLA2 | Q9P2R7 | MAGEA11 | Homo sapiens | P43364 | 32296183 | |
|
Intra
|
SUCLA2 | Q9P2R7 | OPTN | Homo sapiens | Q96CV9 | 32814053 | |
|
Intra
|
SUCLA2 | Q9P2R7 | OPTN | Homo sapiens | Q96CV9 | 32814053 | |
|
Intra
|
SUCLA2 | Q9P2R7 | OPTN | Homo sapiens | Q96CV9 | 32814053 | |
|
Intra
|
SUCLA2 | Q9P2R7 | SEC23A | Homo sapiens | Q15436 | 32296183 | |
|
Intra
|
SUCLA2 | Q9P2R7 | SEC23A | Homo sapiens | Q15436 | 32296183 | |
|
Intra
|
SUCLA2 | Q9P2R7 | MAL2 | Homo sapiens | Q969L2 | 32296183 | |
|
Intra
|
SUCLA2 | Q9P2R7 | MAL2 | Homo sapiens | Q969L2 | 32296183 |
Related Diseases
| Diseases | Alias | |
|---|---|---|
| Mitochondrial Dna Depletion Syndrome 5 |
|
|
| Mitochondrial Dna Depletion Syndrome |
|
|
| Gaba Aminotransferase Deficiency |
|
|
| Mitochondrial Dna Depletion Syndrome 6 |
|
|
| Combined Oxidative Phosphorylation Deficiency 13 |
|
|
| Progressive External Ophthalmoplegia With Mitochondrial Dna Deletions, Autosomal Dominant 4 |
|
|
| Mitochondrial Dna Depletion Syndrome 9 |
|
|
| Infantile Cerebellar-Retinal Degeneration |
|
|
| Fumarase Deficiency |
|
|
| Combined Malonic And Methylmalonic Aciduria |
|
|
| Methylmalonic Aciduria, Cbla Type |
|
|
| Methylmalonic Aciduria Due To Methylmalonyl-Coa Mutase Deficiency |
|
|
| Mitochondrial Metabolism Disease |
|
|
| Mitochondrial Dna Depletion Syndrome 7 |
|
|
| 3-Methylglutaconic Aciduria With Deafness, Encephalopathy, And Leigh-Like Syndrome |
|
|
| 3-Methylglutaconic Aciduria, Type Iv |
|
|
| Kearns-Sayre Syndrome |
|
|
| 3-Methylglutaconic Aciduria, Type Iii |
|
|
| Mitochondrial Dna Depletion Syndrome 4a |
|
|
| Combined Oxidative Phosphorylation Deficiency 1 |
|
|
| Methylmalonic Acidemia |
|
|
| Developmental And Epileptic Encephalopathy 7 |
|
|
| Sensory Ataxic Neuropathy, Dysarthria, And Ophthalmoparesis |
|
|
| Organic Acidemia |
|
|
| Chronic Progressive External Ophthalmoplegia |
|
|
| 3-Methylglutaconic Aciduria |
|
|
| Neuropathy, Ataxia, And Retinitis Pigmentosa |
|
|
| Fatal Infantile Cardioencephalomyopathy Due To Cytochrome C Oxidase Deficiency |
|
|
| Mitochondrial Encephalomyopathy |
|
|
| Mitochondrial Myopathy |
|
|
| Leigh Syndrome |
|
|
| Lactic Acidosis |
|
Orthologs Information
| Species | Symbol | Source | ID |
|---|---|---|---|
| Felis catus | SUCLA2 | VGNC | VGNC:102338 |
| Canis familiaris | SUCLA2 | VGNC | VGNC:54998 |
| Rattus norvegicus | SUCLA2 | RGD | RGD:1309397 |
| Macaca mulatta | SUCLA2 | VGNC | VGNC:103872 |
| Mus musculus | SUCLA2 | MGD | MGI:1306775 |
| Bos taurus | SUCLA2 | VGNC | VGNC:35455 |
| Others | SUCLA2 | NCBI |