Biochemistry of Amino Acids - A. Majster 1961

Disorders of Amino Acid Metabolism in Certain Pathological States
Phenylalanine and Tyrosine Metabolism
Albinism and Pheochromocytoma

Albinism is a congenital anomaly of Tyrosine METABOLISM characterized by the failure of melanin Biosynthesis and inherited in an autosomal recessive manner. In this condition, The conversion of DOPA (dihydroxyphenylalanine) to melanin is apparently blocked. A defect localized at an earlier stage of tyrosine metabolism is unlikely, since albinism shows no evidence of impaired adrenaline biosynthesis (cf. [179]).

Pheochromocytomas are adrenaline- and noradrenaline-producing tumors that develop from the Cells of The adrenal medulla or the sympathetic Nervous system ganglia [180]. The symptoms associated with this disorder can be explained by the hypersecretion of adrenaline and noradrenaline; these Hormones are readily detected in the urine of such patients [181]. Radical surgical removal of the tumor eliminates this syndrome. These tumors, which secrete large amounts of adrenaline and noradrenaline, can serve as a useful model for studying The Mechanism of biosynthesis of these hormones.



Last update: 06/08/2026

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