SUCLA2
entrez
8803
canonical
uniprot
Q9P2R7
canonical
ensembl
ENSG00000136143
resolver
ensp
ENSP00000494360
resolver
genesymbol
SUCLA2
resolver
hgnc
HGNC:11448
resolver
uniprot
B2RDE7
resolver
uniprot
E5KS60
resolver
uniprot
O95194
resolver
uniprot
Q5T9Q4
resolver
uniprot
Q5T9Q6
resolver
uniprot
Q9NV21
resolver
uniprot
Q9NVP7
resolver
uniprot_entry
E5KS60_HUMAN
resolver
uniprot_entry
SUCB1_HUMAN
resolver
uniprot_sec
B2RDE7
resolver
uniprot_sec
O95194
resolver
uniprot_sec
Q5T9Q4
resolver
uniprot_sec
Q5T9Q6
resolver
uniprot_sec
Q9NV21
resolver
uniprot_sec
Q9NVP7
resolver
activity_regulation
ACTIVITY REGULATION: Inhibited by itaconate. {ECO:0000269|PubMed:34492704}.
uniprot
proteins
amino_acid_sequence
MAASMFYGRLVAVATLRNHRPRTAQRAAAQVLGSSGLFNNHGLQVQQQQQRNLSLHEYMSMELLQEAGVSVPKGYVAKSPDEAYAIAKKLGSKDVVIKAQVLAGGRGKGTFESGLKGGVKIVFSPEEAKAVSSQMIGKKLFTKQTGEKGRICNQVLVCERKYPRREYYFAITMERSFQGPVLIGSSHGGVNIEDVAAESPEAIIKEPIDIEEGIKKEQALQLAQKMGFPPNIVESAAENMVKLYSLFLKYDATMIEINPMVEDSDGAVLCMDAKINFDSNSAYRQKKIFDLQDWTQEDERDKDAAKANLNYIGLDGNIGCLVNGAGLAMATMDIIKLHGGTPANFLDVGGGATVHQVTEAFKLITSDKKVLAILVNIFGGIMRCDVIAQGIVMAVKDLEIKIPVVVRLQGTRVDDAKALIADSGLKILACDDLDEAARMVVKLSEIVTLAKQAHVDVKFQLPI
uniprot
proteins
disease_involvement
DISEASE: Mitochondrial DNA depletion syndrome 5 (MTDPS5) [MIM:612073]: A disorder due to mitochondrial dysfunction. It is characterized by infantile onset of hypotonia, neurologic deterioration, a hyperkinetic-dystonic movement disorder, external ophthalmoplegia, deafness, variable renal tubular dysfunction, and mild methylmalonic aciduria in some patients. {ECO:0000269|PubMed:15877282, ECO:0000269|PubMed:17287286, ECO:0000269|PubMed:17301081, ECO:0000269|PubMed:23759946}. 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
6.2.1.5
uniprot
proteins
ec_number
6.2.1.9
uniprot
proteins
function
FUNCTION: ATP-specific succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of ATP and thus represents the only step of substrate-level phosphorylation in the TCA (PubMed:15877282, PubMed:34492704, PubMed:40108300). The beta subunit provides nucleotide specificity of the enzyme and binds the substrate succinate, while the binding sites for coenzyme A and phosphate are found in the alpha subunit (By similarity). Also able to act as an ATP-specific itaconyl- and malyl-CoA synthetase (PubMed:40108300). {ECO:0000255|HAMAP-Rule:MF_03220, ECO:0000269|PubMed:15877282, ECO:0000269|PubMed:34492704, ECO:0000269|PubMed:40108300}.
uniprot
proteins
mass_dalton
50317
uniprot
proteins
pathway_participation
PATHWAY: Carbohydrate metabolism; tricarboxylic acid cycle; succinate from succinyl-CoA (ligase route): step 1/1. {ECO:0000255|HAMAP-Rule:MF_03220, ECO:0000305|PubMed:15877282}.
uniprot
proteins
protein_family
ATP-specific subunit beta subfamily
uniprot
proteins
protein_family
Succinate/malate CoA ligase beta subunit family
uniprot
proteins
pubmed
10508479
uniprot
proteins
pubmed
10727444
uniprot
proteins
pubmed
14643893
uniprot
proteins
pubmed
14702039
uniprot
proteins
pubmed
15057823
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
15592455
uniprot
proteins
pubmed
15877282
uniprot
proteins
pubmed
17287286
uniprot
proteins
pubmed
17301081
uniprot
proteins
pubmed
19608861
uniprot
proteins
pubmed
21269460
uniprot
proteins
pubmed
23759946
uniprot
proteins
pubmed
24275569
uniprot
proteins
pubmed
25944712
uniprot
proteins
pubmed
34492704
uniprot
proteins
pubmed
40108300
uniprot
proteins
pubmed
9765291
uniprot
proteins
sequence_length
463
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion {ECO:0000255|HAMAP-Rule:MF_03220, ECO:0000269|PubMed:15877282, ECO:0000269|PubMed:17287286, ECO:0000269|PubMed:25944712}.
uniprot
proteins