SUCLG1

Protein identityUniProt · HPA

UniProt accession

P53597

Gene

SUCLG1

Ensembl

ENSG00000163541

Chromosome

2

Canonical length

346 aa

Protein class

Citric acid cycle related proteins, Disease related genes, Enzymes, Human disease related genes, Metabolic proteins, Plasma proteins, Potential drug targets, Predicted intracellular proteins

Subcellular location

Plasma membrane,Mitochondria,Cytosol

OverviewNCBI Gene

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]

Canonical amino-acid sequenceUniProt

346 residues, UniProt reviewed canonical sequence.

>P53597|SUCLG1
     1  MTATLAAAAD IATMVSGSSG LAAARLLSRS FLLPQNGIRH CSYTASRQHL YVDKNTKIIC
    61  QGFTGKQGTF HSQQALEYGT KLVGGTTPGK GGQTHLGLPV FNTVKEAKEQ TGATASVIYV
   121  PPPFAAAAIN EAIEAEIPLV VCITEGIPQQ DMVRVKHKLL RQEKTRLIGP NCPGVINPGE
   181  CKIGIMPGHI HKKGRIGIVS RSGTLTYEAV HQTTQVGLGQ SLCVGIGGDP FNGTDFIDCL
   241  EIFLNDSATE GIILIGEIGG NAEENAAEFL KQHNSGPNSK PVVSFIAGLT APPGRRMGHA
   301  GAIIAGGKGG AKEKISALQS AGVVVSMSPA QLGTTIYKEF EKRKML

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against SUCLG1 can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability

Intracellular

Secreted

No

Transmembrane segments

0

Mean surface accessibility (rSASA)

0.29

Highest tissue expression

517 nTPM

Expression across tissuesHPA

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about SUCLG1.

Disease | AllUniProt

Conditions SUCLG1 is implicated in, by any mechanism.

• Mitochondrial DNA depletion syndrome 9 (MTDPS9) MIM:245400

Disease | GeneticClinVar

32 pathogenic / likely-pathogenic of 375 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

• Mitochondrial DNA depletion syndrome 9
• Melanoma

Genetic constraint and essentialitygnomAD · DepMap

Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.

gnomAD LOEUF (loss-of-function intolerance)

0.88

gnomAD pLI

0

gnomAD missense Z

-0.22

DepMap mean gene effect

-0.2

DepMap dependency class

selective

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 6% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).

OntologyGO

Biological processes

• succinyl-CoA catabolic process
• tricarboxylic acid cycle

Molecular functions

• nucleotide binding
• RNA binding
• succinate-CoA ligase (ADP-forming) activity
• succinate-CoA ligase (GDP-forming) activity
• succinate-CoA ligase activity

Cellular components

• mitochondrial matrix
• mitochondrion
• succinate-CoA ligase complex (ADP-forming)
• succinate-CoA ligase complex (GDP-forming)

Protein domainsUniProt · Pfam · InterPro

• CoA-binding
• ATP-citrate synthase/succinyl-CoA ligase, C-terminal domain
• Succinyl-CoA synthetase-like
• ATP-citrate lyase/succinyl-CoA ligase, active site
• ATP-citrate lyase/succinyl-CoA ligase, conserved site
• NAD(P)-binding domain superfamily
• CoA-ligase
• CoA binding domain
• Succinyl-CoA ligase, alpha subunit

KeywordsUniProt

• Acetylation
• Ligase
• Mitochondrion
• Nucleotide-binding
• Primary mitochondrial disease
• Transit peptide
• Tricarboxylic acid cycle

InteractionsUniProt · HPA

Protein binding partners of SUCLG1 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

• SUCLG2