Article
Mechanistic studies of human molybdopterin synthase reaction and characterization of mutants identified in group B patients of molybdenum cofactor deficiency.
The Journal of biological chemistry - 11 Jul 2003
Leimkuhler Silke, Freuer Andrea, Araujo Jose Angel Santamaria, Rajagopalan K V, Mendel Ralf R
Abstract excerpt
Biosynthesis of the molybdenum cofactor involves the initial formation of precursor Z, its subsequent conversion to molybdopterin (MPT) by MPT synthase, and attachment of molybdenum to the dithiolene moiety of MPT. The sulfur used for the formation of the dithiolene group of MPT exists in the form of a thiocarboxylate group at the C terminus of the smaller subunit of MPT synthase. Human MPT synthase contains the...
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