LMPD Database

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LMP004255

Record overview

LMPD IDLMP004255
Gene ID8802
SpeciesHomo sapiens (Human)
Gene Namesuccinate-CoA ligase, alpha subunit
Gene SymbolSUCLG1
SynonymsGALPHA; MTDPS9; SUCLA1
Alternate namessuccinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial; SCS-alpha; succinyl-CoA synthetase subunit alpha; succinyl-CoA ligase [GDP-forming] subunit alpha, mitochondrial
Chromosome2
Map Location2p11.2
EC Number6.2.1.4
SummaryThis 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]
OrthologsView orthologs and multiple alignments for SUCLG1

Proteins

succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial precursor
Refseq ID:NP_003840
Protein GI:109452591
UniProt ID:P53597
mRNA ID:NM_003849
Length:346
RefSeq Status:REVIEWED
MTATLAAAADIATMVSGSSGLAAARLLSRSFLLPQNGIRHCSYTASRQHLYVDKNTKIICQGFTGKQGTFHSQQALEYGTKLVGGTTPGKGGQTHLGLPV
FNTVKEAKEQTGATASVIYVPPPFAAAAINEAIEAEIPLVVCITEGIPQQDMVRVKHKLLRQEKTRLIGPNCPGVINPGECKIGIMPGHIHKKGRIGIVS
RSGTLTYEAVHQTTQVGLGQSLCVGIGGDPFNGTDFIDCLEIFLNDSATEGIILIGEIGGNAEENAAEFLKQHNSGPNSKPVVSFIAGLTAPPGRRMGHA
GAIIAGGKGGAKEKISALQSAGVVVSMSPAQLGTTIYKEFEKRKML
 
transit_peptide: 1..34
inference: protein motif:TargetP:1.1
calculated_mol_wt: 3336
peptide sequence: 
MTATLAAAADIATMVSGSSGLAAARLLSRSFLLP

mat_peptide: 35..346
product: succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial
calculated_mol_wt: 32932
peptide sequence: 
QNGIRHCSYTASRQHLYVDKNTKIICQGFTGKQGTFHSQQALEYGTKLVGGTTPGKGGQTHLGLPVFNTVKEAKEQTGATASVIYVPPPFAAAAINEAIE
AEIPLVVCITEGIPQQDMVRVKHKLLRQEKTRLIGPNCPGVINPGECKIGIMPGHIHKKGRIGIVSRSGTLTYEAVHQTTQVGLGQSLCVGIGGDPFNGT
DFIDCLEIFLNDSATEGIILIGEIGGNAEENAAEFLKQHNSGPNSKPVVSFIAGLTAPPGRRMGHAGAIIAGGKGGAKEKISALQSAGVVVSMSPAQLGT
TIYKEFEKRKML