Gene/Proteome Database (LMPD)
LMPD ID
LMP005947
Gene ID
Species
Homo sapiens (Human)
Gene Name
succinate-CoA ligase, ADP-forming, beta subunit
Gene Symbol
Synonyms
A-BETA; MTDPS5; SCS-betaA
Chromosome
13
Map Location
13q12.2-q13.3
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]
Orthologs
Proteins
| succinyl-CoA ligase [ADP-forming] subunit beta, mitochondrial precursor | |
|---|---|
| Refseq ID | NP_003841 |
| Protein GI | 11321583 |
| UniProt ID | Q9Y4T0 |
| mRNA ID | NM_003850 |
| Length | 463 |
| RefSeq Status | REVIEWED |
| MAASMFYGRLVAVATLRNHRPRTAQRAAAQVLGSSGLFNNHGLQVQQQQQRNLSLHEYMSMELLQEAGVSVPKGYVAKSPDEAYAIAKKLGSKDVVIKAQVLAGGRGKGTFESGLKGGVKIVFSPEEAKAVSSQMIGKKLFTKQTGEKGRICNQVLVCERKYPRREYYFAITMERSFQGPVLIGSSHGGVNIEDVAAESPEAIIKEPIDIEEGIKKEQALQLAQKMGFPPNIVESAAENMVKLYSLFLKYDATMIEINPMVEDSDGAVLCMDAKINFDSNSAYRQKKIFDLQDWTQEDERDKDAAKANLNYIGLDGNIGCLVNGAGLAMATMDIIKLHGGTPANFLDVGGGATVHQVTEAFKLITSDKKVLAILVNIFGGIMRCDVIAQGIVMAVKDLEIKIPVVVRLQGTRVDDAKALIADSGLKILACDDLDEAARMVVKLSEIVTLAKQAHVDVKFQLPI | |
| transit_peptide: 1..52 calculated_mol_wt: 5751 peptide sequence: MAASMFYGRLVAVATLRNHRPRTAQRAAAQVLGSSGLFNNHGLQVQQQQQRN mat_peptide: 53..463 product: succinyl-CoA ligase [ADP-forming] subunit beta, mitochondrial calculated_mol_wt: 44585 peptide sequence: LSLHEYMSMELLQEAGVSVPKGYVAKSPDEAYAIAKKLGSKDVVIKAQVLAGGRGKGTFESGLKGGVKIVFSPEEAKAVSSQMIGKKLFTKQTGEKGRICNQVLVCERKYPRREYYFAITMERSFQGPVLIGSSHGGVNIEDVAAESPEAIIKEPIDIEEGIKKEQALQLAQKMGFPPNIVESAAENMVKLYSLFLKYDATMIEINPMVEDSDGAVLCMDAKINFDSNSAYRQKKIFDLQDWTQEDERDKDAAKANLNYIGLDGNIGCLVNGAGLAMATMDIIKLHGGTPANFLDVGGGATVHQVTEAFKLITSDKKVLAILVNIFGGIMRCDVIAQGIVMAVKDLEIKIPVVVRLQGTRVDDAKALIADSGLKILACDDLDEAARMVVKLSEIVTLAKQAHVDVKFQLPI | |
Gene Information
Entrez Gene ID
Gene Name
succinate-CoA ligase, ADP-forming, beta subunit
Gene Symbol
Species
Homo sapiens
Gene Ontology (GO Annotations)
| GO ID | Source | Type | Description |
|---|---|---|---|
| GO:0005739 | IDA:HPA | C | mitochondrion |
| GO:0005524 | IEA:InterPro | F | ATP binding |
| GO:0046872 | IEA:InterPro | F | metal ion binding |
| GO:0004775 | IEA:Ensembl | F | succinate-CoA ligase (ADP-forming) activity |
| GO:0006105 | IEA:Ensembl | P | succinate metabolic process |
| GO:0006104 | IEA:Ensembl | P | succinyl-CoA metabolic process |
| GO:0006099 | IEA:Ensembl | P | tricarboxylic acid cycle |
KEGG Pathway Links
Domain Information
InterPro Annotations
| Accession | Description |
|---|---|
| IPR005811 | ATP-citrate lyase/succinyl-CoA ligase |
| IPR011761 | ATP-grasp fold |
| IPR013815 | ATP-grasp fold, subdomain 1 |
| IPR013816 | ATP-grasp fold, subdomain 2 |
| IPR013650 | ATP-grasp fold, succinyl-CoA synthetase-type |
| IPR005809 | Succinyl-CoA synthetase, beta subunit |
| IPR017866 | Succinyl-CoA synthetase, beta subunit, conserved site |
| IPR016102 | Succinyl-CoA synthetase-like |
UniProt Annotations
Entry Information
Gene Name
succinate-CoA ligase, ADP-forming, beta subunit
Protein Entry
SUCB1_HUMAN
UniProt ID
Species
Human
Identical and Related Proteins
Unique RefSeq proteins for LMP005947 (as displayed in Record Overview)
| Protein GI | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| 11321583 | RefSeq | NP_003841 | 463 | succinyl-CoA ligase [ADP-forming] subunit beta, mitochondrial precursor |
Identical Sequences to LMP005947 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:11321583 | GenBank | ADP91754.1 | 463 | mitochondrial succinyl-CoA ligase [ADP-forming] subunit beta [Homo sapiens] |
| GI:11321583 | GenBank | ADP91759.1 | 463 | mitochondrial succinyl-CoA ligase [ADP-forming] subunit beta [Homo sapiens] |
| GI:11321583 | GenBank | ADP91762.1 | 463 | mitochondrial succinyl-CoA ligase [ADP-forming] subunit beta [Homo sapiens] |
| GI:11321583 | GenBank | ADP91765.1 | 463 | mitochondrial succinyl-CoA ligase [ADP-forming] subunit beta [Homo sapiens] |
| GI:11321583 | GenBank | AHD77770.1 | 463 | Sequence 23899 from patent US 8586006 |
| GI:11321583 | GenBank | AIC50209.1 | 463 | SUCLA2, partial [synthetic construct] |
Related Sequences to LMP005947 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:11321583 | DBBJ | BAG37894.1 | 463 | unnamed protein product [Homo sapiens] |
| GI:11321583 | GenBank | EAX08777.1 | 463 | succinate-CoA ligase, ADP-forming, beta subunit, isoform CRA_c [Homo sapiens] |
| GI:11321583 | GenBank | ADP91734.1 | 463 | mitochondrial succinyl-CoA ligase [ADP-forming] subunit beta [Homo sapiens] |
| GI:11321583 | GenBank | ADP91735.1 | 463 | mitochondrial succinyl-CoA ligase [ADP-forming] subunit beta [Homo sapiens] |
| GI:11321583 | GenBank | ADP91736.1 | 463 | mitochondrial succinyl-CoA ligase [ADP-forming] subunit beta [Homo sapiens] |
| GI:11321583 | RefSeq | XP_009425211.1 | 463 | PREDICTED: succinyl-CoA ligase [ADP-forming] subunit beta, mitochondrial [Pan troglodytes] |