4ap8 | pdb_00004ap8
From Proteopedia
Crystal structure of human Molybdopterin synthase catalytic subunit (MOCS2B)
| ||||||||||||
Structural highlights
Disease[MOC2B_HUMAN] Molybdenum cofactor deficiency type B (MOCOD type B) [MIM:252150]: Autosomal recessive disease which leads to the pleiotropic loss of all molybdoenzyme activities and is characterized by severe neurological damage, neonatal seizures and early childhood death. Note=The disease is caused by mutations affecting the gene represented in this entry. Function[MOC2B_HUMAN] Catalytic subunit of the molybdopterin synthase complex, a complex that catalyzes the conversion of precursor Z into molybdopterin. Acts by mediating the incorporation of 2 sulfur atoms from thiocarboxylated MOCS2A into precursor Z to generate a dithiolene group.[1] [2] References
| ||||||||||||||||||||
This page was last modified 05:41, 25 August 2022.