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MGP003696

Record overview

MGPD IDMGP003696
Gene ID8803
SpeciesHomo sapiens (Human)
Gene Namesuccinate-CoA ligase, ADP-forming, beta subunit
Gene Symbol SUCLA2
SynonymsA-BETA; MTDPS5; SCS-betaA;
Alternate namessuccinyl-CoA ligase [ADP-forming] subunit beta, mitochondrial; ATP-specific succinyl-CoA synthetase subunit beta; ATP-specific succinyl-CoA synthetase, beta subunit; mitochondrial succinyl-CoA ligase [ADP-forming] subunit beta; renal carcinoma antigen NY-REN-39; succinyl-CoA synthetase beta-A chain;
Chromosome13
Map Location13q14.2
EC Number6.2.1.5
SummarySuccinyl-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]
OrthologsView orthologs and multiple alignments for SUCLA2

Proteins

succinyl-CoA ligase [ADP-forming] subunit beta, mitochondrial precursor
Refseq ID:NP_003841
Protein GI:11321583
UniProt ID:Q9P2R7
mRNA ID:NM_003850
Length:463
RefSeq Status:
MAASMFYGRLVAVATLRNHRPRTAQRAAAQVLGSSGLFNNHGLQVQQQQQRNLSLHEYMSMELLQEAGVSVPKGYVAKSPDEAYAIAKKLGSKDVVIKAQ
VLAGGRGKGTFESGLKGGVKIVFSPEEAKAVSSQMIGKKLFTKQTGEKGRICNQVLVCERKYPRREYYFAITMERSFQGPVLIGSSHGGVNIEDVAAESP
EAIIKEPIDIEEGIKKEQALQLAQKMGFPPNIVESAAENMVKLYSLFLKYDATMIEINPMVEDSDGAVLCMDAKINFDSNSAYRQKKIFDLQDWTQEDER
DKDAAKANLNYIGLDGNIGCLVNGAGLAMATMDIIKLHGGTPANFLDVGGGATVHQVTEAFKLITSDKKVLAILVNIFGGIMRCDVIAQGIVMAVKDLEI
KIPVVVRLQGTRVDDAKALIADSGLKILACDDLDEAARMVVKLSEIVTLAKQAHVDVKFQLPI
 
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: 
LSLHEYMSMELLQEAGVSVPKGYVAKSPDEAYAIAKKLGSKDVVIKAQVLAGGRGKGTFESGLKGGVKIVFSPEEAKAVSSQMIGKKLFTKQTGEKGRIC
NQVLVCERKYPRREYYFAITMERSFQGPVLIGSSHGGVNIEDVAAESPEAIIKEPIDIEEGIKKEQALQLAQKMGFPPNIVESAAENMVKLYSLFLKYDA
TMIEINPMVEDSDGAVLCMDAKINFDSNSAYRQKKIFDLQDWTQEDERDKDAAKANLNYIGLDGNIGCLVNGAGLAMATMDIIKLHGGTPANFLDVGGGA
TVHQVTEAFKLITSDKKVLAILVNIFGGIMRCDVIAQGIVMAVKDLEIKIPVVVRLQGTRVDDAKALIADSGLKILACDDLDEAARMVVKLSEIVTLAKQ
AHVDVKFQLPI
 
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