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Regulatory news: Nulibry (fosdenopterin) approved to reduce the risk of mortality in patients with molybdenum cofactor deficiency type A: FDA approval summary

Molybdenum cofactor deficiency (MoCD) type A is a very rare, fatal, autosomal recessive disease with an estimated U.S. prevalence of approximately 50 patients, primarily under 10 years of age. It is caused by pathogenic variants in the MOCS1 gene that lead to deficiency of cyclic pyranopterin monophosphate (cPMP), an intermediate in the biosynthetic pathway of molybdenum cofactor (MoCo), an essential cofactor of certain enzymes. cPMP deficiency causes accumulation of toxic metabolites including sulfites, taurine, thiosulfate, and S-sulfocysteine (SSC). Patients experience intractable seizures, failure to thrive, feeding difficulties, axial hypotonia, limb hypertonia, and significantly delayed neurocognitive and motor skills. Most patients die in the first years of life with a median survival of 36 months. Treatment thus far has focused on managing symptoms. Fosdenopterin is a chemically synthesized form of endogenous cPMP. It treats MoCD type A by replacing the deficient cPMP substrate and permitting the biosynthesis of MoCo, enabling activation of MoCo-dependent enzymes and the reduction of accumulated sulfites. Fosdenopterin is administered as an intravenous infusion once daily based on body weight.