Differentiation and developmental origin of cerebellar granule neuron ectopia in protein O-mannose UDP-N-acetylglucosaminyl transferase 1 knockout mice.

Differentiation and developmental origin of cerebellar granule neuron ectopia in protein O-mannose UDP-N-acetylglucosaminyl transferase 1 knockout mice. Research Abstract Details 

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Differentiation and developmental origin of cerebellar granule neuron ectopia in protein O-mannose UDP-N-acetylglucosaminyl transferase 1 knockout mice. Abstract Text:

X Li,P Zhang,Y Yang,Y Xiong,Y Qi,H Hu,

The cerebellar cortex of protein O-mannose UDP-N-acetylglucosaminyl transferase 1 (POMGnT1) knockout mice contains discrete clusters of granule neurons that fail to migrate from the external germinal layer (EGL) to the internal granule cell layer (IGL). To test the hypothesis that the breaches in the pial basement membrane and glia limitans contribute to the formation of such heterotopias, POMGnT1 deficient mice were used to examine the mechanisms underlying these migration defects. The basement membrane, glia limitans, and granule neuron development were assessed with protein markers and immunofluorescent microscopy. Further, the integrity of the pial basement membrane, and granule neuron differentiation state were assessed by electron microscopy. Localized breaches in pial basement membrane and disruptions in the glia limitans were strongly associated with ectopia of EGL cells. In such ectopias, Bergmann glia fibers were retracted and disorganized with very few protruded into the ectopic area. Thus, migration failure was correlated with a compromised Bergmann glia scaffold. Nevertheless, the ectopic EGL cells showed characteristics of differentiated granule neurons and formed synapses with mossy fibers. Altogether, these results suggest that pial basement membrane breaches and glia limitans disruptions are the underlying causes of cerebellar granule neuron ectopia in POMGnT1 knockout mice. Moreover, migration into the IGL is not required for granule cell acquisition of certain differentiated characteristics.

Differentiation and developmental origin of cerebellar granule neuron ectopia in protein O-mannose UDP-N-acetylglucosaminyl transferase 1 knockout mice. Publishing Authors By Initials

X Li,P Zhang,Y Yang,Y Xiong,Y Qi,H Hu,

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Differentiation and developmental origin of cerebellar granule neuron ectopia in protein O-mannose UDP-N-acetylglucosaminyl transferase 1 knockout mice. Journal Published:

PUBLICATION TYPE: Journal Article

Journal: Neuroscience

VOLUME: 152

Page Numbers: 391-406

Journal Abbreviation: Neuroscience

ISSN: 0306-4522

DAY: 17

MONTH: 07

YEAR: 2007

Differentiation and developmental origin of cerebellar granule neuron ectopia in protein O-mannose UDP-N-acetylglucosaminyl transferase 1 knockout mice. Information

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LANGUAGE: eng

NlmUniqueID: 7605074

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AFFILIATION: Department of Neuroscience and Physiology, Upstate Medical University, Syracuse, NY 13210, USA; Department of Neurology, No. 2 Hospital Affiliated to Chongqing Medical University, Chongqing 400010, PR China.

Country: United States

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