title: Neuropeptide Y expression in phaeochromocytomas: relative absence in tumours from patients with von Hippel-Lindau syndrome.
abstract: 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.
authors.affiliation: Clinical Neurocardiology Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA. s.cleary@murdoch.edu.au
authors.forename: Susannah
authors.initials: S
authors.lastname: Cleary
authors.forename: Jacqueline K
authors.initials: JK
authors.lastname: Phillips
authors.forename: Thanh-Truc
authors.initials: TT
authors.lastname: Huynh
authors.forename: Karel
authors.initials: K
authors.lastname: Pacak
authors.forename: Abdel G
authors.initials: AG
authors.lastname: Elkahloun
authors.forename: Jennifer
authors.initials: J
authors.lastname: Barb
authors.forename: Robert A
authors.initials: RA
authors.lastname: Worrell
authors.forename: David S
authors.initials: DS
authors.lastname: Goldstein
authors.forename: Graeme
authors.initials: G
authors.lastname: Eisenhofer
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000737:chemistry*; D000328:Adult; D000368:Aged; D000704:Analysis of Variance; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D016014:Linear Models; D008297:Male; D008875:Middle Aged; D018813:Multiple Endocrine Neoplasia Type 2a / Q000737:chemistry; D009478:Neuropeptide Y / Q000032:analysis* / Q000235:genetics; D010625:Phenylethanolamine N-Methyltransferase / Q000032:analysis; D010673:Pheochromocytoma / Q000737:chemistry*; D012333:RNA, Messenger / Q000032:analysis; D020133:Reverse Transcriptase Polymerase Chain Reaction; D006623:von Hippel-Lindau Disease / Q000378:metabolism*
chemical_list: D009478:Neuropeptide Y; D012333:RNA, Messenger; D010625:Phenylethanolamine N-Methyltransferase