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

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

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

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

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

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

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

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

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

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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.

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