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Spt4 selectively regulates the expression of C9orf72 sense and antisense mutant transcripts

Targeting three defects with one strategyThe neurodegenerative diseases amyotrophic lateral sclerosis and frontotemporal dementia are most commonly caused by a mutation in the C9orf72 gene. The mutation is an expanded hexanucleotide repeat in a noncoding region. The expanded repeat produces sense an... Full description

Journal Title: Science August 12, 2016, Vol.353(6300), pp.708-712
Main Author: Kramer, Nicholas
Other Authors: Carlomagno, Yari , Zhang, Yong-Jie , Almeida, Sandra , Cook, Casey , Gendron, Tania , Prudencio, Mercedes , Van Blitterswijk, Marka , Belzil, Veronique , Couthouis, Julien , Paul, Joseph , Goodman, Lindsey , Daughrity, Lillian , Chew, Jeannie , Garrett, Aliesha , Pregent, Luc , Jansen-West, Karen , Tabassian, Lilia , Rademakers, Rosa , Boylan
Format: Electronic Article Electronic Article
Language: English
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ID: ISSN: 0036-8075 ; DOI: 10.1126/science.aaf7791
Link: http://search.proquest.com/docview/1835661975/?pq-origsite=primo
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title: Spt4 selectively regulates the expression of C9orf72 sense and antisense mutant transcripts
format: Article
creator:
  • Kramer, Nicholas
  • Carlomagno, Yari
  • Zhang, Yong-Jie
  • Almeida, Sandra
  • Cook, Casey
  • Gendron, Tania
  • Prudencio, Mercedes
  • Van Blitterswijk, Marka
  • Belzil, Veronique
  • Couthouis, Julien
  • Paul, Joseph
  • Goodman, Lindsey
  • Daughrity, Lillian
  • Chew, Jeannie
  • Garrett, Aliesha
  • Pregent, Luc
  • Jansen-West, Karen
  • Tabassian, Lilia
  • Rademakers, Rosa
  • Boylan
subjects:
  • Elongation
  • Mathematical Models
  • Genes
  • Mutations
  • Ribonucleic Acids
  • Proteins
  • Patients
  • Degeneration
  • Miscellaneous Sciences (So)
  • (An)
ispartof: Science, August 12, 2016, Vol.353(6300), pp.708-712
description: Targeting three defects with one strategyThe neurodegenerative diseases amyotrophic lateral sclerosis and frontotemporal dementia are most commonly caused by a mutation in the C9orf72 gene. The mutation is an expanded hexanucleotide repeat in a noncoding region. The expanded repeat produces sense and antisense RNA transcripts, which accumulate in patient cells and appear to sequester RNA-binding proteins. The sense and antisense transcripts are also translated into dipeptide repeat proteins, which are aggregation-prone and accumulate in the brain and spinal cord. Last, loss of function from reduced expression of C9orf72 in neurons and glia could contribute to the disease. Kramer et al. targeted both sense and antisense repeats by blocking a single gene called SPT4, which mitigated degeneration in human cells by reducing all three types of pathologies.Science, this issue p. 708 An expanded hexanucleotide repeat in C9orf72 causes amyotrophic lateral sclerosis and frontotemporal...
language: eng
source:
identifier: ISSN: 0036-8075 ; DOI: 10.1126/science.aaf7791
fulltext: no_fulltext
issn:
  • 00368075
  • 0036-8075
url: Link


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titleSpt4 selectively regulates the expression of C9orf72 sense and antisense mutant transcripts
creatorKramer, Nicholas ; Carlomagno, Yari ; Zhang, Yong-Jie ; Almeida, Sandra ; Cook, Casey ; Gendron, Tania ; Prudencio, Mercedes ; Van Blitterswijk, Marka ; Belzil, Veronique ; Couthouis, Julien ; Paul, Joseph ; Goodman, Lindsey ; Daughrity, Lillian ; Chew, Jeannie ; Garrett, Aliesha ; Pregent, Luc ; Jansen-West, Karen ; Tabassian, Lilia ; Rademakers, Rosa ; Boylan
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ispartofScience, August 12, 2016, Vol.353(6300), pp.708-712
identifierISSN: 0036-8075 ; DOI: 10.1126/science.aaf7791
subjectElongation ; Mathematical Models ; Genes ; Mutations ; Ribonucleic Acids ; Proteins ; Patients ; Degeneration ; Miscellaneous Sciences (So) ; (An)
descriptionTargeting three defects with one strategyThe neurodegenerative diseases amyotrophic lateral sclerosis and frontotemporal dementia are most commonly caused by a mutation in the C9orf72 gene. The mutation is an expanded hexanucleotide repeat in a noncoding region. The expanded repeat produces sense and antisense RNA transcripts, which accumulate in patient cells and appear to sequester RNA-binding proteins. The sense and antisense transcripts are also translated into dipeptide repeat proteins, which are aggregation-prone and accumulate in the brain and spinal cord. Last, loss of function from reduced expression of C9orf72 in neurons and glia could contribute to the disease. Kramer et al. targeted both sense and antisense repeats by blocking a single gene called SPT4, which mitigated degeneration in human cells by reducing all three types of pathologies.Science, this issue p. 708 An expanded hexanucleotide repeat in C9orf72 causes amyotrophic lateral sclerosis and frontotemporal...
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titleSpt4 selectively regulates the expression of C9orf72 sense and antisense mutant transcripts
descriptionTargeting three defects with one strategyThe neurodegenerative diseases amyotrophic lateral sclerosis and frontotemporal dementia are most commonly caused by a mutation in the C9orf72 gene. The mutation is an expanded hexanucleotide repeat in a noncoding region. The expanded repeat produces sense and antisense RNA transcripts, which accumulate in patient cells and appear to sequester RNA-binding proteins. The sense and antisense transcripts are also translated into dipeptide repeat proteins, which are aggregation-prone and accumulate in the brain and spinal cord. Last, loss of function from reduced expression of C9orf72 in neurons and glia could contribute to the disease. Kramer et al. targeted both sense and antisense repeats by blocking a single gene called SPT4, which mitigated degeneration in human cells by reducing all three types of pathologies.Science, this issue p. 708 An expanded hexanucleotide repeat in C9orf72 causes amyotrophic lateral sclerosis and frontotemporal...
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titleSpt4 selectively regulates the expression of C9orf72 sense and antisense mutant transcripts
authorKramer, Nicholas ; Carlomagno, Yari ; Zhang, Yong-Jie ; Almeida, Sandra ; Cook, Casey ; Gendron, Tania ; Prudencio, Mercedes ; Van Blitterswijk, Marka ; Belzil, Veronique ; Couthouis, Julien ; Paul, Joseph ; Goodman, Lindsey ; Daughrity, Lillian ; Chew, Jeannie ; Garrett, Aliesha ; Pregent, Luc ; Jansen-West, Karen ; Tabassian, Lilia ; Rademakers, Rosa ; Boylan
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abstractTargeting three defects with one strategyThe neurodegenerative diseases amyotrophic lateral sclerosis and frontotemporal dementia are most commonly caused by a mutation in the C9orf72 gene. The mutation is an expanded hexanucleotide repeat in a noncoding region. The expanded repeat produces sense and antisense RNA transcripts, which accumulate in patient cells and appear to sequester RNA-binding proteins. The sense and antisense transcripts are also translated into dipeptide repeat proteins, which are aggregation-prone and accumulate in the brain and spinal cord. Last, loss of function from reduced expression of C9orf72 in neurons and glia could contribute to the disease. Kramer et al. targeted both sense and antisense repeats by blocking a single gene called SPT4, which mitigated degeneration in human cells by reducing all three types of pathologies.Science, this issue p. 708 An expanded hexanucleotide repeat in C9orf72 causes amyotrophic lateral sclerosis and frontotemporal...
doi10.1126/science.aaf7791
urlhttp://search.proquest.com/docview/1835661975/
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date2016-08-12