Basal ganglia dopamine loss due to defect in purine recycling

Several rare inherited disorders have provided valuable experiments of nature highlighting specific biological processes of particular importance to the survival or function of midbrain dopamine neurons. In both humans and mice, deficiency of hypoxanthine-guanine phosphoribosyl transferase (HPRT) is...

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Main Authors:	Kiyoshi Egami, Silaja Yitta, Suhail Kasim, J. Chris Lewers, Rosalinda C. Roberts, Mohamed Lehar, H.A. Jinnah
Format:	Article
Language:	English
Published:	Elsevier 2007-05-01
Series:	Neurobiology of Disease
Online Access:	http://www.sciencedirect.com/science/article/pii/S0969996107000265

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spelling	doaj-8ddd82f90ce24391a860402a2830be482021-03-20T04:54:17ZengElsevierNeurobiology of Disease1095-953X2007-05-01262396407Basal ganglia dopamine loss due to defect in purine recyclingKiyoshi Egami0Silaja Yitta1Suhail Kasim2J. Chris Lewers3Rosalinda C. Roberts4Mohamed Lehar5H.A. Jinnah6Department of Neurology, Meyer Room 6-181, 600 North Wolfe Street, Johns Hopkins Hospital, Baltimore, MD 21287, USADepartment of Neurology, Meyer Room 6-181, 600 North Wolfe Street, Johns Hopkins Hospital, Baltimore, MD 21287, USADepartment of Neurology, Meyer Room 6-181, 600 North Wolfe Street, Johns Hopkins Hospital, Baltimore, MD 21287, USADepartment of Neurology, Meyer Room 6-181, 600 North Wolfe Street, Johns Hopkins Hospital, Baltimore, MD 21287, USAMaryland Psychiatric Research Center, Department of Psychiatry, University of Maryland School of Medicine, Baltimore, MD 21228, USADepartment of Otolaryngology, Head and Neck Surgery, Johns Hopkins University, Baltimore, MD 21287, USADepartment of Neurology, Meyer Room 6-181, 600 North Wolfe Street, Johns Hopkins Hospital, Baltimore, MD 21287, USA; Corresponding author. Fax: +1 410 502 6737.Several rare inherited disorders have provided valuable experiments of nature highlighting specific biological processes of particular importance to the survival or function of midbrain dopamine neurons. In both humans and mice, deficiency of hypoxanthine-guanine phosphoribosyl transferase (HPRT) is associated with profound loss of striatal dopamine, with relative preservation of other neurotransmitters. In the current studies of knockout mice, no morphological signs of abnormal development or degeneration were found in an exhaustive battery that included stereological and morphometric measures of midbrain dopamine neurons, electron microscopic studies of striatal axons and terminals, and stains for degeneration or gliosis. A novel culture model involving HPRT-deficient dopaminergic neurons also exhibited significant loss of dopamine without a morphological correlate. These results suggest that dopamine loss in HPRT deficiency has a biochemical rather than anatomical basis and imply that purine recycling to be a biochemical process of particular importance to the function of dopaminergic neurons.http://www.sciencedirect.com/science/article/pii/S0969996107000265
collection	DOAJ
language	English
format	Article
sources	DOAJ
author	Kiyoshi Egami Silaja Yitta Suhail Kasim J. Chris Lewers Rosalinda C. Roberts Mohamed Lehar H.A. Jinnah
spellingShingle	Kiyoshi Egami Silaja Yitta Suhail Kasim J. Chris Lewers Rosalinda C. Roberts Mohamed Lehar H.A. Jinnah Basal ganglia dopamine loss due to defect in purine recycling Neurobiology of Disease
author_facet	Kiyoshi Egami Silaja Yitta Suhail Kasim J. Chris Lewers Rosalinda C. Roberts Mohamed Lehar H.A. Jinnah
author_sort	Kiyoshi Egami
title	Basal ganglia dopamine loss due to defect in purine recycling
title_short	Basal ganglia dopamine loss due to defect in purine recycling
title_full	Basal ganglia dopamine loss due to defect in purine recycling
title_fullStr	Basal ganglia dopamine loss due to defect in purine recycling
title_full_unstemmed	Basal ganglia dopamine loss due to defect in purine recycling
title_sort	basal ganglia dopamine loss due to defect in purine recycling
publisher	Elsevier
series	Neurobiology of Disease
issn	1095-953X
publishDate	2007-05-01
description	Several rare inherited disorders have provided valuable experiments of nature highlighting specific biological processes of particular importance to the survival or function of midbrain dopamine neurons. In both humans and mice, deficiency of hypoxanthine-guanine phosphoribosyl transferase (HPRT) is associated with profound loss of striatal dopamine, with relative preservation of other neurotransmitters. In the current studies of knockout mice, no morphological signs of abnormal development or degeneration were found in an exhaustive battery that included stereological and morphometric measures of midbrain dopamine neurons, electron microscopic studies of striatal axons and terminals, and stains for degeneration or gliosis. A novel culture model involving HPRT-deficient dopaminergic neurons also exhibited significant loss of dopamine without a morphological correlate. These results suggest that dopamine loss in HPRT deficiency has a biochemical rather than anatomical basis and imply that purine recycling to be a biochemical process of particular importance to the function of dopaminergic neurons.
url	http://www.sciencedirect.com/science/article/pii/S0969996107000265
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Basal ganglia dopamine loss due to defect in purine recycling

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