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Regional distribution of the phenylalanine‐sensitive ATP‐sulphurylase in brain
Author(s) -
Matsuo K.,
Hommes F.
Publication year - 1987
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/bf01799489
Subject(s) - phenylalanine , central nervous system , pathogenesis , distribution (mathematics) , medicine , hyperphenylalaninemia , endocrinology , biology , chemistry , biochemistry , amino acid , mathematical analysis , mathematics
The regional distribution of the phenylalanine‐sensitive ATP‐sulphurylase in fetal calf brain coincides with demyelinated lesions observed in the central nervous systems of untreated PKU patients. This would be expected if this species of ATP‐sulphurylase played a role in the pathogenesis of brain dysfunction in the untreated or poorly controlled phenylketonuria patient.