SUCLG1
entrez
8802
canonical
uniprot
P53597
canonical
ensembl
ENSG00000163541
resolver
ensp
ENSP00000377446
resolver
enst
ENST00000393868
resolver
genesymbol
SUCLG1
resolver
hgnc
HGNC:11449
resolver
uniprot
Q9BWB0
resolver
uniprot
Q9UNP6
resolver
uniprot_entry
SUCA_HUMAN
resolver
uniprot_sec
Q9BWB0
resolver
uniprot_sec
Q9UNP6
resolver
activity_regulation
ACTIVITY REGULATION: Inhibited by itaconate. {ECO:0000269|PubMed:34492704}.
uniprot
proteins
amino_acid_sequence
MTATLAAAADIATMVSGSSGLAAARLLSRSFLLPQNGIRHCSYTASRQHLYVDKNTKIICQGFTGKQGTFHSQQALEYGTKLVGGTTPGKGGQTHLGLPVFNTVKEAKEQTGATASVIYVPPPFAAAAINEAIEAEIPLVVCITEGIPQQDMVRVKHKLLRQEKTRLIGPNCPGVINPGECKIGIMPGHIHKKGRIGIVSRSGTLTYEAVHQTTQVGLGQSLCVGIGGDPFNGTDFIDCLEIFLNDSATEGIILIGEIGGNAEENAAEFLKQHNSGPNSKPVVSFIAGLTAPPGRRMGHAGAIIAGGKGGAKEKISALQSAGVVVSMSPAQLGTTIYKEFEKRKML
uniprot
proteins
disease_involvement
DISEASE: Mitochondrial DNA depletion syndrome 9 (MTDPS9) [MIM:245400]: A severe disorder due to mitochondrial dysfunction. It is characterized by infantile onset of hypotonia, lactic acidosis, severe psychomotor retardation, progressive neurologic deterioration, and excretion of methylmalonic acid. {ECO:0000269|PubMed:17668387, ECO:0000269|PubMed:19526370, ECO:0000269|PubMed:20453710, ECO:0000269|PubMed:20693550}. Note=The disease is caused by variants affecting the gene represented in this entry.
uniprot
proteins
ec_number
6.2.1.-
uniprot
proteins
ec_number
6.2.1.4
uniprot
proteins
ec_number
6.2.1.5
uniprot
proteins
ec_number
6.2.1.9
uniprot
proteins
function
FUNCTION: Succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of either ATP or GTP and thus represents the only step of substrate-level phosphorylation in the TCA (PubMed:34492704, PubMed:40108300). The alpha subunit of the enzyme binds the substrates coenzyme A and phosphate, while succinate binding and specificity for either ATP or GTP is provided by different beta subunits (By similarity). Also able to act as an itaconyl- and malyl-CoA synthetase (PubMed:40108300). {ECO:0000255|HAMAP-Rule:MF_03222, ECO:0000269|PubMed:34492704, ECO:0000269|PubMed:40108300}.
uniprot
proteins
mass_dalton
36250
uniprot
proteins
pathway_participation
PATHWAY: Carbohydrate metabolism; tricarboxylic acid cycle; succinate from succinyl-CoA (ligase route): step 1/1. {ECO:0000255|HAMAP-Rule:MF_03222}.
uniprot
proteins
protein_family
Succinate/malate CoA ligase alpha subunit family
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
15815621
uniprot
proteins
pubmed
17668387
uniprot
proteins
pubmed
19526370
uniprot
proteins
pubmed
19608861
uniprot
proteins
pubmed
20453710
uniprot
proteins
pubmed
20693550
uniprot
proteins
pubmed
21269460
uniprot
proteins
pubmed
24275569
uniprot
proteins
pubmed
25944712
uniprot
proteins
pubmed
26566883
uniprot
proteins
pubmed
34492704
uniprot
proteins
pubmed
40108300
uniprot
proteins
pubmed
9128182
uniprot
proteins
sequence_length
346
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion {ECO:0000255|HAMAP-Rule:MF_03222}.
uniprot
proteins