Target intelligence / Profile preview

Molybdenum cofactor biosynthesis protein 1 (MOCS1)

Target: MOCS1
Molecular classification: Enzyme

Overview

Molybdenum cofactor biosynthesis protein 1 is a key enzyme involved in the conserved pathway that leads to the biological activation of molybdenum. It catalyzes the conversion of 5'-GTP into cyclic pyranopterin monophosphate (cPMP), an essential intermediate in molybdenum cofactor biosynthesis. Defects in MOCS1 cause type A molybdenum cofactor deficiency, a severe metabolic disorder.

Other names: MIG11MOCODMOCODAmolybdenum cofactor synthesis 1

Biological functions

Molybdenum cofactor biosynthesiscPMP formation

Disease associations

Molybdenum cofactor deficiencyNeurological disorders

Safety considerations

Severe neurological impairment due to accumulation of toxic metabolites

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