Abstract
Pheochromocytomas in multiple endocrine neoplasia type
2 (MEN-2) express phenylethanolamine N-methyltransferase (PNMT),
the enzyme that catalyzes conversion of norepinephrine to epinephrine,
whereas those in von HippelLindau (VHL) syndrome do not. Consequently,
pheochromocytomas in MEN-2 produce epinephrine, whereas
those in VHL syndrome produce mainly norepinephrine. This study
examined whether transcription factors known to regulate expression
of PNMT explain the different tumor phenotypes in these syndromes.
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