Reactome | DLST transfers glutaryl to CoA

DLST transfers glutaryl to CoA

Stable Identifier

R-HSA-9858590

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

ReviewStatus

5/5

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Dihydrolipoyl succinyl transferase (DLST), the E2 component of the 2-oxoadipate dehydrogenase metabolon, self-associates into a cubic octamer of homotrimers. The trimer appears to be the minimal conformation for optimal activity. It transfers the glutaryl moiety bound to one of its N6-dihydrolipoyllysine residues onto the substrate CoA (Nemeria et al., 2017). Mutations in DLST can lead to benign pheochromocytomas and paragangliomas (PGL7; MIM:618475; Remacha et al., 2019).

Literature References

PubMed ID	Title	Journal	Year
29191460	The mitochondrial 2-oxoadipate and 2-oxoglutarate dehydrogenase complexes share their E2 and E3 components for their function and both generate reactive oxygen species Zhang, X, Jordan, F, Yang, L, Nareddy, PR, Nemeria, NS, Gerfen, G, Szostak, M	Free Radic Biol Med	2018
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