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

created [InstanceEdit:9859030] Stephan, Ralf, 2024-01-15
dbId 9859066
displayName Dihydrolipoyl succinyl transferase (DLST), the E2 component ...
schemaClass Summation
text 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).
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