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Ataxia and Purkinje cell degeneration in mice lacking the CAMTA1 transcription factor.

Members of the calmodulin-binding transcription activator (CAMTA) family of proteins function as calcium-sensitive regulators of gene expression in multicellular organisms ranging from plants to humans. Here, we show that global or nervous system deletion of CAMTA1 in mice causes severe ataxia with... Full description

Journal Title: Proceedings of the National Academy of Sciences of the United States of America August 5, 2014, Vol.111(31), pp.11521-11526
Main Author: Long, Chengzu
Other Authors: Grueter, Chad E , Song, Kunhua , Qin, Song , Qi, Xiaoxia , Kong, Y Megan , Shelton, John M , Richardson, James A , Zhang, Chun-Li , Bassel-Duby, Rhonda , Olson, Eric N
Format: Electronic Article Electronic Article
Language: English
Subjects:
ID: E-ISSN: 1091-6490 ; DOI: 10.1073/pnas.1411251111
Link: http://search.proquest.com/docview/1551828087/?pq-origsite=primo
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title: Ataxia and Purkinje cell degeneration in mice lacking the CAMTA1 transcription factor.
format: Article
creator:
  • Long, Chengzu
  • Grueter, Chad E
  • Song, Kunhua
  • Qin, Song
  • Qi, Xiaoxia
  • Kong, Y Megan
  • Shelton, John M
  • Richardson, James A
  • Zhang, Chun-Li
  • Bassel-Duby, Rhonda
  • Olson, Eric N
subjects:
  • At Rich Sequence–Metabolism
  • Animals–Pathology
  • Ataxia–Physiopathology
  • Base Sequence–Deficiency
  • Binding Sites–Metabolism
  • Calcium-Binding Proteins–Metabolism
  • Gene Expression Regulation–Genetics
  • Integrases–Metabolism
  • Inverted Repeat Sequences–Genetics
  • Male–Metabolism
  • Mice–Pathology
  • Mice, Knockout–Deficiency
  • Molecular Sequence Data–Metabolism
  • Motor Activity–Deficiency
  • Nestin–Metabolism
  • Nucleotide Motifs–Metabolism
  • Protein Multimerization–Metabolism
  • Purkinje Cells–Metabolism
  • Trans-Activators–Metabolism
  • Transcription Factors
ispartof: Proceedings of the National Academy of Sciences of the United States of America, August 5, 2014, Vol.111(31), pp.11521-11526
description: Members of the calmodulin-binding transcription activator (CAMTA) family of proteins function as calcium-sensitive regulators of gene expression in multicellular organisms ranging from plants to humans. Here, we show that global or nervous system deletion of CAMTA1 in mice causes severe ataxia with Purkinje cell degeneration and cerebellar atrophy, partially resembling the consequences of haploinsufficiency of the human CAMTA1 locus. Gene-expression analysis identified a large collection of neuronal genes that were dysregulated in the brains of CAMTA1-mutant mice, and elucidation of a consensus sequence for binding of CAMTA proteins to DNA revealed the association of CAMTA-binding sites with many of these genes. We conclude that CAMTA1 plays an essential role in the control of Purkinje cell function and survival. CAMTA1-mutant mice provide a model to study the molecular mechanisms of neurodegenerative diseases and for screening potential therapeutic interventions for such disorders.
language: eng
source:
identifier: E-ISSN: 1091-6490 ; DOI: 10.1073/pnas.1411251111
fulltext: fulltext
issn:
  • 10916490
  • 1091-6490
url: Link


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titleAtaxia and Purkinje cell degeneration in mice lacking the CAMTA1 transcription factor.
creatorLong, Chengzu ; Grueter, Chad E ; Song, Kunhua ; Qin, Song ; Qi, Xiaoxia ; Kong, Y Megan ; Shelton, John M ; Richardson, James A ; Zhang, Chun-Li ; Bassel-Duby, Rhonda ; Olson, Eric N
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identifierE-ISSN: 1091-6490 ; DOI: 10.1073/pnas.1411251111
subjectAt Rich Sequence–Metabolism ; Animals–Pathology ; Ataxia–Physiopathology ; Base Sequence–Deficiency ; Binding Sites–Metabolism ; Calcium-Binding Proteins–Metabolism ; Gene Expression Regulation–Genetics ; Integrases–Metabolism ; Inverted Repeat Sequences–Genetics ; Male–Metabolism ; Mice–Pathology ; Mice, Knockout–Deficiency ; Molecular Sequence Data–Metabolism ; Motor Activity–Deficiency ; Nestin–Metabolism ; Nucleotide Motifs–Metabolism ; Protein Multimerization–Metabolism ; Purkinje Cells–Metabolism ; Trans-Activators–Metabolism ; Transcription Factors
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descriptionMembers of the calmodulin-binding transcription activator (CAMTA) family of proteins function as calcium-sensitive regulators of gene expression in multicellular organisms ranging from plants to humans. Here, we show that global or nervous system deletion of CAMTA1 in mice causes severe ataxia with Purkinje cell degeneration and cerebellar atrophy, partially resembling the consequences of haploinsufficiency of the human CAMTA1 locus. Gene-expression analysis identified a large collection of neuronal genes that were dysregulated in the brains of CAMTA1-mutant mice, and elucidation of a consensus sequence for binding of CAMTA proteins to DNA revealed the association of CAMTA-binding sites with many of these genes. We conclude that CAMTA1 plays an essential role in the control of Purkinje cell function and survival. CAMTA1-mutant mice provide a model to study the molecular mechanisms of neurodegenerative diseases and for screening potential therapeutic interventions for such disorders.
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titleAtaxia and Purkinje cell degeneration in mice lacking the CAMTA1 transcription factor.
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titleAtaxia and Purkinje cell degeneration in mice lacking the CAMTA1 transcription factor.
authorLong, Chengzu ; Grueter, Chad E ; Song, Kunhua ; Qin, Song ; Qi, Xiaoxia ; Kong, Y Megan ; Shelton, John M ; Richardson, James A ; Zhang, Chun-Li ; Bassel-Duby, Rhonda ; Olson, Eric N
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