Dihydrolipoyl succinyl transferase (DLST), the E2 component ...

created [InstanceEdit:9853770] Stephan, Ralf, 2023-11-21
dbId 9853764
displayName Dihydrolipoyl succinyl transferase (DLST), the E2 component ...
schemaClass Summation
text 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).
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