A novel migration-related gene product, doublecortin, in neuronal migration disorder of fetuses and infants with Zellweger syndrome.

A novel migration-related gene product, doublecortin, in neuronal migration disorder of fetuses and infants with Zellweger syndrome. Research Abstract Details 

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A novel migration-related gene product, doublecortin, in neuronal migration disorder of fetuses and infants with Zellweger syndrome. Abstract Text:

Immunohistochemical expression of doublecortin (DCX), KIAA0369 (KIA) and LIS1 proteins as well as nestin and vimentin in the cerebral cortices of six patients with Zellweger syndrome (ZS), aged 19 gestational weeks (GW) to 8 months, was compared with that in nine controls, aged 12 GW to 8 months. DCX immunoreactivity was apparently reduced in ZS, particularly in the cortical plate of fetuses, and in the subependymal foci of heterotopic neurons of the infants. Reduced expression of DCX in ZS was confirmed by Western blot analysis. On the other hand, neuronal expression of nestin was high in the cortical plate, migrating cells of the white matter and germinal cells in the ventricular zone in fetuses with ZS. Immunoreactivities for KIA, LIS1 and vimentin in ZS were comparable to those of controls. Reduced expression of DCX may be responsible for the neuronal migration disorder, and increased expression of nestin may be another evidence for delayed neuronal maturation in ZS.

A novel migration-related gene product, doublecortin, in neuronal migration disorder of fetuses and infants with Zellweger syndrome. Publishing Authors By Initials

For similar digestive system diseases: liver diseases: zellweger syndrome research abstracts see: digestive system diseases: liver diseases: zellweger syndrome research

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A novel migration-related gene product, doublecortin, in neuronal migration disorder of fetuses and infants with Zellweger syndrome. Journal Published:

PUBLICATION TYPE: Journal Article

Journal: Acta neuropathologica

VOLUME: 100

Page Numbers: 168-73

Journal Abbreviation: Acta Neuropathol.

ISSN: 0001-6322

DAY: 6

MONTH: Aug

YEAR: 2000

A novel migration-related gene product, doublecortin, in neuronal migration disorder of fetuses and infants with Zellweger syndrome. Information

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LANGUAGE: eng

NlmUniqueID: 412041

A novel migration-related gene product, doublecortin, in neuronal migration disorder of fetuses and infants with Zellweger syndrome. Keywords Mesh Terms:

KEYWORDS: Zellweger Syndrome

MESH TERMS: metabolism

Chemical & Substance for Abstract: A novel migration-related gene product, doublecortin, in neuronal migration disorder of fetuses and infants with Zellweger syndrome. Information

Substance Name: PAFAH1B1 protein, human

Registry Number: EC 3.1.1.47

Grant and Affiliation Information for A novel migration-related gene product, doublecortin, in neuronal migration disorder of fetuses and infants with Zellweger syndrome.

AFFILIATION: Department of Mental Retardation, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan.

Country: GERMANY

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MEDLINETA: Acta Neuropathol

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