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, Sakamoto, T, Inazawa, J, Ohta, S, Nakagawa, S, Matuda, S, Takase, C	Eur J Biochem	1994
30929736	Recurrent Germline DLST Mutations in Individuals with Multiple Pheochromocytomas and Paragangliomas Richter, S, Torres-Pérez, R, Remacha, L, Mahoney, CE, Luque, RM, Herráez, B, Robledo, M, Cianchetta, G, Eisenhofer, G, García-Uriarte, Ó, Maestre, L, Moreno-Rengel, C, Pita, G, Gálvez, MÁ, Coloma, J, Lahera, M, Letón, R, Currás-Freixes, M, Honrado, E, Rodríguez-Antona, C, Aller, J, Urioste, M, Calsina, B, Pirman, D, Montero-Conde, C, Cascón, A, Smolen, GA, Llorca, O	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 Zambo, Z, Jordan, F, Adam-Vizi, V, Novaček, J, Ambrus, A, Szabo, E, Polak, M, Hubert, A, Ozohanics, O, Nagy, B	Biochim Biophys Acta Gen Subj	2021