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¹ Clinical Neurocardiology Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, USA
² Division of Health Sciences, Murdoch University and Western Australian Biomedical Research Institute, Perth, Western Australia, Australia
³ Reproductive Biology and Medicine Branch, National Institute of Child Health and Human Development,
⁴ Genome Technology Branch, National Human Genome Research Institute,
⁵ Mathematical and Statistical Computing Laboratory, Center for Information Technology and
⁶ Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA

(Requests for offprints should be addressed to S Cleary, School of Veterinary and Biomedical Sciences, Murdoch University, Perth, Western Australia 6150, Australia; Email: s.cleary{at}murdoch.edu.au)

Phaeochromocytomas are rare neuroendocrine tumours that produce catecholamines and numerous secretory proteins and peptides, including neuropeptide Y (NPY), a vasoactive peptide with influences on blood pressure. The production of catecholamines and NPY by phaeochromocytomas is highly variable. This study examined influences of hereditary factors and differences in catecholamine production on tumour expression of NPY, as assessed by quantitative PCR, enzyme immunoassay and immunohistochemistry. Phaeochromocytomas included hereditary adrenaline-producing tumours (adrenergic phenotype) in multiple endocrine neoplasia type 2 (MEN 2), predominantly noradrenaline-producing tumours (noradrenergic phenotype) in von Hippel–Lindau (VHL) syndrome, and other adrenergic and noradrenergic tumours where there was no clear hereditary syndrome. NPY levels in phaeochromocytomas from VHL patients were lower (P<0.0001) than in those from MEN 2 patients for both mRNA (84-fold difference) and the peptide (99-fold difference). These findings were supported by immunohistochemistry. NPY levels were also lower in VHL tumours than in those where there was no hereditary syndrome. Relative absence of expression of NPY in phaeochromocytomas from VHL patients when compared with other groups appears to be largely independent of differences in catecholamine production and is consistent with a unique phenotype in VHL syndrome.

This article has been cited by other articles:

				G. Eisenhofer, T.-T. Huynh, A. Elkahloun, J. C. Morris, G. Bratslavsky, W. M. Linehan, Z. Zhuang, B. M. Balgley, C. S. Lee, M. Mannelli, et al. Differential expression of the regulated catecholamine secretory pathway in different hereditary forms of pheochromocytoma Am J Physiol Endocrinol Metab, November 1, 2008; 295(5): E1223 - E1233. [Abstract] [Full Text] [PDF]