Reactome | DLST transfers succinyl to CoA

DLST transfers succinyl to CoA

Stable Identifier

R-HSA-9853512

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

(R)-N(6)-(S(8)-succinyldihydrolipoyl)-L-lysyl-(2-oxoglutarate dehydrogenase complex component E2) + CoA => (R)-N(6)-dihydrolipoyl-L-lysyl-(2-oxoglutarate dehydrogenase complex component E2) + succinyl-CoA

ReviewStatus

5/5

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Dihydrolipoyl succinyl transferase (DLST), the E2 component of the 2-oxoglutarate dehydrogenase metabolon, self-associates into a cubic octamer of homotrimers. The trimer appears to be the minimal conformation for optimal activity. It transfers the succinyl moiety bound to one of its N6-dihydrolipoyllysine residues onto the substrate CoA (Nakano et al., 1994; Nagy et al., 2021). Mutations in DLST can lead to benign pheochromocytomas and paragangliomas (PGL7; MIM:618475; Remacha et al., 2019).

Literature References

PubMed ID	Title	Journal	Year
8076640	Isolation, characterization and structural organization of the gene and pseudogene for the dihydrolipoamide succinyltransferase component of the human 2-oxoglutarate dehydrogenase complex Nakano, K, Takase, C, Sakamoto, T, Nakagawa, S, Inazawa, J, Ohta, S, Matuda, S	Eur J Biochem	1994
30929736	Recurrent Germline DLST Mutations in Individuals with Multiple Pheochromocytomas and Paragangliomas Remacha, L, Pirman, D, Mahoney, CE, Coloma, J, Calsina, B, Currás-Freixes, M, Letón, R, Torres-Pérez, R, Richter, S, Pita, G, Herráez, B, Cianchetta, G, Honrado, E, Maestre, L, Urioste, M, Aller, J, García-Uriarte, Ó, Gálvez, MÁ, Luque, RM, Lahera, M, Moreno-Rengel, C, Eisenhofer, G, Montero-Conde, C, Rodríguez-Antona, C, Llorca, O, Smolen, GA, Robledo, M, Cascón, A	Am J Hum Genet	2019
33684457	Structure of the dihydrolipoamide succinyltransferase (E2) component of the human alpha-ketoglutarate dehydrogenase complex (hKGDHc) revealed by cryo-EM and cross-linking mass spectrometry: Implications for the overall hKGDHc structure Nagy, B, Polak, M, Ozohanics, O, Zambo, Z, Szabo, E, Hubert, A, Jordan, F, Novaček, J, Adam-Vizi, V, Ambrus, A	Biochim Biophys Acta Gen Subj	2021