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Loss of the golgin GM130 causes Golgi disruption, Purkinje neuron loss, and ataxia in mice.

The Golgi apparatus lies at the heart of the secretory pathway where it is required for secretory trafficking and cargo modification. Disruption of Golgi architecture and function has been widely observed in neurodegenerative disease, but whether Golgi dysfunction is causal with regard to the neurod... Full description

Journal Title: Proceedings of the National Academy of Sciences of the United States of America January 10, 2017, Vol.114(2), pp.346-351
Main Author: Liu, Chunyi
Other Authors: Mei, Mei , Li, Qiuling , Roboti, Peristera , Pang, Qianqian , Ying, Zhengzhou , Gao, Fei , Lowe, Martin , Bao, Shilai
Format: Electronic Article Electronic Article
Language: English
Subjects:
ID: E-ISSN: 1091-6490 ; DOI: 10.1073/pnas.1608576114
Link: http://search.proquest.com/docview/1853746279/?pq-origsite=primo
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recordid: proquest1853746279
title: Loss of the golgin GM130 causes Golgi disruption, Purkinje neuron loss, and ataxia in mice.
format: Article
creator:
  • Liu, Chunyi
  • Mei, Mei
  • Li, Qiuling
  • Roboti, Peristera
  • Pang, Qianqian
  • Ying, Zhengzhou
  • Gao, Fei
  • Lowe, Martin
  • Bao, Shilai
subjects:
  • Animals–Metabolism
  • Ataxia–Metabolism
  • Autoantigens–Metabolism
  • Dendrites–Metabolism
  • Female–Metabolism
  • Golgi Apparatus–Metabolism
  • Male–Metabolism
  • Membrane Proteins–Physiology
  • Mice–Metabolism
  • Mice, Inbred C57bl–Physiology
  • Neurodegenerative Diseases–Physiology
  • Neurons–Physiology
  • Protein Transport–Physiology
  • Purkinje Cells–Physiology
  • Secretory Pathway–Physiology
  • Autoantigens
  • Golgin Subfamily A Member 2
  • Membrane Proteins
  • Gm130
  • Golgi Apparatus
  • Purkinje Cell
  • Ataxia
  • Polarized Secretion
ispartof: Proceedings of the National Academy of Sciences of the United States of America, January 10, 2017, Vol.114(2), pp.346-351
description: The Golgi apparatus lies at the heart of the secretory pathway where it is required for secretory trafficking and cargo modification. Disruption of Golgi architecture and function has been widely observed in neurodegenerative disease, but whether Golgi dysfunction is causal with regard to the neurodegenerative process, or is simply a manifestation of neuronal death, remains unclear. Here we report that targeted loss of the golgin GM130 leads to a profound neurological phenotype in mice. Global KO of mouse GM130 results in developmental delay, severe ataxia, and postnatal death. We further show that selective deletion of GM130 in neurons causes fragmentation and defective positioning of the Golgi apparatus, impaired secretory trafficking, and dendritic atrophy in Purkinje cells. These cellular defects manifest as reduced cerebellar size and Purkinje cell number, leading to ataxia. Purkinje cell loss and ataxia first appear during postnatal development but progressively worsen with age. Our data therefore indicate that targeted disruption of the mammalian Golgi apparatus and secretory traffic results in neuronal degeneration in vivo, supporting the view that Golgi dysfunction can play a causative role in neurodegeneration.
language: eng
source:
identifier: E-ISSN: 1091-6490 ; DOI: 10.1073/pnas.1608576114
fulltext: fulltext
issn:
  • 10916490
  • 1091-6490
url: Link


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titleLoss of the golgin GM130 causes Golgi disruption, Purkinje neuron loss, and ataxia in mice.
creatorLiu, Chunyi ; Mei, Mei ; Li, Qiuling ; Roboti, Peristera ; Pang, Qianqian ; Ying, Zhengzhou ; Gao, Fei ; Lowe, Martin ; Bao, Shilai
contributorLiu, Chunyi (correspondence author) ; Liu, Chunyi (record owner)
ispartofProceedings of the National Academy of Sciences of the United States of America, January 10, 2017, Vol.114(2), pp.346-351
identifierE-ISSN: 1091-6490 ; DOI: 10.1073/pnas.1608576114
subjectAnimals–Metabolism ; Ataxia–Metabolism ; Autoantigens–Metabolism ; Dendrites–Metabolism ; Female–Metabolism ; Golgi Apparatus–Metabolism ; Male–Metabolism ; Membrane Proteins–Physiology ; Mice–Metabolism ; Mice, Inbred C57bl–Physiology ; Neurodegenerative Diseases–Physiology ; Neurons–Physiology ; Protein Transport–Physiology ; Purkinje Cells–Physiology ; Secretory Pathway–Physiology ; Autoantigens ; Golgin Subfamily A Member 2 ; Membrane Proteins ; Gm130 ; Golgi Apparatus ; Purkinje Cell ; Ataxia ; Polarized Secretion
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descriptionThe Golgi apparatus lies at the heart of the secretory pathway where it is required for secretory trafficking and cargo modification. Disruption of Golgi architecture and function has been widely observed in neurodegenerative disease, but whether Golgi dysfunction is causal with regard to the neurodegenerative process, or is simply a manifestation of neuronal death, remains unclear. Here we report that targeted loss of the golgin GM130 leads to a profound neurological phenotype in mice. Global KO of mouse GM130 results in developmental delay, severe ataxia, and postnatal death. We further show that selective deletion of GM130 in neurons causes fragmentation and defective positioning of the Golgi apparatus, impaired secretory trafficking, and dendritic atrophy in Purkinje cells. These cellular defects manifest as reduced cerebellar size and Purkinje cell number, leading to ataxia. Purkinje cell loss and ataxia first appear during postnatal development but progressively worsen with age. Our data therefore indicate that targeted disruption of the mammalian Golgi apparatus and secretory traffic results in neuronal degeneration in vivo, supporting the view that Golgi dysfunction can play a causative role in neurodegeneration.
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titleLoss of the golgin GM130 causes Golgi disruption, Purkinje neuron loss, and ataxia in mice.
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titleLoss of the golgin GM130 causes Golgi disruption, Purkinje neuron loss, and ataxia in mice.
authorLiu, Chunyi ; Mei, Mei ; Li, Qiuling ; Roboti, Peristera ; Pang, Qianqian ; Ying, Zhengzhou ; Gao, Fei ; Lowe, Martin ; Bao, Shilai
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