8v0j

Structural highlights

Disease

LIAS_HUMAN Lipoic acid synthetase deficiency. The disease is caused by variants affecting the gene represented in this entry.

Function

LIAS_HUMAN Catalyzes the radical-mediated insertion of two sulfur atoms into the C-6 and C-8 positions of the octanoyl moiety bound to the lipoyl domains of lipoate-dependent enzymes, thereby converting the octanoylated domains into lipoylated derivatives.[HAMAP-Rule:MF_03123]