Article
Pheochromocytomas in von Hippel-Lindau syndrome and multiple endocrine neoplasia type 2 display distinct biochemical and clinical phenotypes.
The Journal of clinical endocrinology and metabolism - 1 May 2001
Eisenhofer G, Walther M M, Huynh T T, Li S T, Bornstein S R, Vortmeyer A, Mannelli M, Goldstein D S, Linehan W M, Lenders J W, Pacak K
Abstract excerpt
This study examined the mechanisms linking different biochemical and clinical phenotypes of pheochromocytoma in multiple endocrine neoplasia type 2 (MEN 2) and von Hippel-Lindau (VHL) syndrome to underlying differences in the expression of tyrosine hydroxylase (TH), the rate-limiting enzyme in catecholamine synthesis, and of phenylethanolamine N-methyltransferase (PNMT), the enzyme that converts norepinephrine to...
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