Gene/Proteome Database (LMPD)

LMPD ID
LMP004255
Gene ID
8802
Species
Homo sapiens (Human)
Gene Name
succinate-CoA ligase, alpha subunit
Gene Symbol
SUCLG1
Synonyms
GALPHA; MTDPS9; SUCLA1
Alternate Names
succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial; SCS-alpha; succinyl-CoA synthetase subunit alpha; succinyl-CoA ligase [GDP-forming] subunit alpha, mitochondrial
Chromosome
2
Map Location
2p11.2
EC Number
6.2.1.4
Summary
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]
Orthologs
View 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
MTATLAAAADIATMVSGSSGLAAARLLSRSFLLPQNGIRHCSYTASRQHLYVDKNTKIICQGFTGKQGTFHSQQALEYGTKLVGGTTPGKGGQTHLGLPVFNTVKEAKEQTGATASVIYVPPPFAAAAINEAIEAEIPLVVCITEGIPQQDMVRVKHKLLRQEKTRLIGPNCPGVINPGECKIGIMPGHIHKKGRIGIVSRSGTLTYEAVHQTTQVGLGQSLCVGIGGDPFNGTDFIDCLEIFLNDSATEGIILIGEIGGNAEENAAEFLKQHNSGPNSKPVVSFIAGLTAPPGRRMGHAGAIIAGGKGGAKEKISALQSAGVVVSMSPAQLGTTIYKEFEKRKML
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: QNGIRHCSYTASRQHLYVDKNTKIICQGFTGKQGTFHSQQALEYGTKLVGGTTPGKGGQTHLGLPVFNTVKEAKEQTGATASVIYVPPPFAAAAINEAIEAEIPLVVCITEGIPQQDMVRVKHKLLRQEKTRLIGPNCPGVINPGECKIGIMPGHIHKKGRIGIVSRSGTLTYEAVHQTTQVGLGQSLCVGIGGDPFNGTDFIDCLEIFLNDSATEGIILIGEIGGNAEENAAEFLKQHNSGPNSKPVVSFIAGLTAPPGRRMGHAGAIIAGGKGGAKEKISALQSAGVVVSMSPAQLGTTIYKEFEKRKML

Gene Information

Entrez Gene ID
8802
Gene Name
succinate-CoA ligase, alpha subunit
Gene Symbol
SUCLG1
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0070062 IDA:UniProt C extracellular vesicular exosome
GO:0005743 IEA:Ensembl C mitochondrial inner membrane
GO:0005759 TAS:Reactome C mitochondrial matrix
GO:0005739 TAS:ProtInc C mitochondrion
GO:0045244 IEA:Ensembl C succinate-CoA ligase complex (GDP-forming)
GO:0003878 IEA:InterPro F ATP citrate synthase activity
GO:0019003 IEA:Ensembl F GDP binding
GO:0005525 IEA:UniProtKB-KW F GTP binding
GO:0048037 IEA:InterPro F cofactor binding
GO:0044822 IDA:UniProtKB F poly(A) RNA binding
GO:0004775 IEA:UniProtKB-EC F succinate-CoA ligase (ADP-forming) activity
GO:0004776 IEA:UniProtKB-EC F succinate-CoA ligase (GDP-forming) activity
GO:0044237 TAS:Reactome P cellular metabolic process
GO:0044281 TAS:Reactome P small molecule metabolic process
GO:0006105 IEA:Ensembl P succinate metabolic process
GO:0006104 IEA:Ensembl P succinyl-CoA metabolic process
GO:0006099 TAS:Reactome P tricarboxylic acid cycle

KEGG Pathway Links

KEGG Pathway ID Description
hsa01200 Carbon metabolism
hsa00020 Citrate cycle (TCA cycle)

Domain Information

InterPro Annotations

Accession Description
IPR005811 ATP-citrate lyase/succinyl-CoA ligase
IPR017440 ATP-citrate lyase/succinyl-CoA ligase, active site
IPR003781 CoA-binding
IPR016040 NAD(P)-binding domain
IPR005810 Succinyl-CoA ligase, alpha subunit
IPR016102 Succinyl-CoA synthetase-like

UniProt Annotations

Entry Information

Gene Name
succinate-CoA ligase, alpha subunit
Protein Entry
SUCA_HUMAN
UniProt ID
P53597
Species
Human

Comments

Comment Type Description
Catalytic Activity ATP + succinate + CoA = ADP + phosphate + succinyl-CoA.
Catalytic Activity GTP + succinate + CoA = GDP + phosphate + succinyl-CoA.
Disease Mitochondrial DNA depletion syndrome 9 (MTDPS9) [MIM
Function Catalyzes the ATP- or GTP-dependent ligation of succinate and CoA to form succinyl-CoA. The nature of the beta subunit determines the nucleotide specificity (By similarity).
Pathway Carbohydrate metabolism; tricarboxylic acid cycle.
Sequence Caution Sequence=AAD17940.2; Type=Erroneous initiation; Evidence= ; Sequence=AAH00504.1; Type=Erroneous initiation; Evidence= ; Sequence=CAA92426.1; Type=Erroneous initiation; Evidence= ;
Similarity Belongs to the succinate/malate CoA ligase alpha subunit family.
Subcellular Location Mitochondrion.
Subunit Heterodimer of an alpha and a beta subunit.

Identical and Related Proteins

Unique RefSeq proteins for LMP004255 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
109452591 RefSeq NP_003840 346 succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial precursor

Identical Sequences to LMP004255 proteins

Reference Database Accession Length Protein Name
GI:109452591 GenBank AAE30554.1 346 Sequence 1 from patent US 5965369
GI:109452591 GenBank EAW99558.1 346 succinate-CoA ligase, GDP-forming, alpha subunit [Homo sapiens]
GI:109452591 GenBank ADA18520.1 346 Sequence 344 from patent US 7608413
GI:109452591 GenBank AEN34416.1 346 Sequence 344 from patent US 7998689
GI:109452591 SwissProt P53597.4 346 RecName: Full=Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial; AltName: Full=Succinyl-CoA synthetase subunit alpha; Short=SCS-alpha; Flags: Precursor [Homo sapiens]

Related Sequences to LMP004255 proteins

Reference Database Accession Length Protein Name
GI:109452591 DBBJ BAE87929.1 346 unnamed protein product [Macaca fascicularis]
GI:109452591 EMBL CAH93082.1 346 hypothetical protein [Pongo abelii]
GI:109452591 RefSeq XP_001164971.1 346 PREDICTED: succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial isoform X1 [Pan troglodytes]
GI:109452591 RefSeq NP_001126825.1 346 succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial [Pongo abelii]
GI:109452591 RefSeq NP_001270301.1 346 uncharacterized protein LOC101867049 [Macaca fascicularis]
GI:109452591 RefSeq XP_007968242.1 415 PREDICTED: succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial [Chlorocebus sabaeus]