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Loss of SNAP-25 and rabphilin 3a in sensory-motor cortex in Huntington's disease.

Smith, Ruben LU ; Klein, Pontus LU ; Koc-Schmitz, Yeliz; Waldvogel, Henry J; Faull, Richard L M; Brundin, Patrik LU ; Plomann, Markus and Li, Jia-Yi (2007) In Journal of Neurochemistry 103(1). p.115-123
Abstract
Huntington’s disease (HD) is an autosomal dominant neurodegenerative disorder caused by a CAG-expansion in the gene encoding the protein huntingtin. The disease is characterized by progressive motor disturbances, cognitive defects, dementia, and weight loss. Using western blotting and immunohistochemistry we have assessed the expression levels and patterns of a number of proteins involved in neurotransmitter release in post-mortem frontal cortex samples from 10 HD cases with different disease grades. We report a loss of the soluble N-ethylmaleimide-sensitive factor attachment protein receptor (SNARE) protein, synaptosome-associated protein 25 (SNAP 25) in HD brains of grades I–IV. Moreover, in brains of grade III and IV we found a... (More)
Huntington’s disease (HD) is an autosomal dominant neurodegenerative disorder caused by a CAG-expansion in the gene encoding the protein huntingtin. The disease is characterized by progressive motor disturbances, cognitive defects, dementia, and weight loss. Using western blotting and immunohistochemistry we have assessed the expression levels and patterns of a number of proteins involved in neurotransmitter release in post-mortem frontal cortex samples from 10 HD cases with different disease grades. We report a loss of the soluble N-ethylmaleimide-sensitive factor attachment protein receptor (SNARE) protein, synaptosome-associated protein 25 (SNAP 25) in HD brains of grades I–IV. Moreover, in brains of grade III and IV we found a reduction in rabphilin 3a, a protein involved in vesicle docking and recycling. These losses appear to be specific and not due to a general loss of synapses in the HD cortex. Thus, levels of synaptobrevin II, syntaxin 1, rab3a or synaptophysin are unaltered in the same patient samples. SNAP 25 and rabphilin 3a are crucial for neurotransmitter release. Therefore, we suggest that a deficient pre-synaptic transmitter release may underlie some of the symptoms of HD. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Journal of Neurochemistry
volume
103
issue
1
pages
115 - 123
publisher
Wiley-Blackwell
external identifiers
  • wos:000249949700010
  • scopus:34548626196
ISSN
1471-4159
DOI
10.1111/j.1471-4159.2007.04703.x
language
English
LU publication?
yes
id
bcf36e82-5012-48ff-b8f2-c3def8bb5dcf (old id 607693)
date added to LUP
2007-12-14 16:38:36
date last changed
2017-11-19 04:07:51
@article{bcf36e82-5012-48ff-b8f2-c3def8bb5dcf,
  abstract     = {Huntington’s disease (HD) is an autosomal dominant neurodegenerative disorder caused by a CAG-expansion in the gene encoding the protein huntingtin. The disease is characterized by progressive motor disturbances, cognitive defects, dementia, and weight loss. Using western blotting and immunohistochemistry we have assessed the expression levels and patterns of a number of proteins involved in neurotransmitter release in post-mortem frontal cortex samples from 10 HD cases with different disease grades. We report a loss of the soluble N-ethylmaleimide-sensitive factor attachment protein receptor (SNARE) protein, synaptosome-associated protein 25 (SNAP 25) in HD brains of grades I–IV. Moreover, in brains of grade III and IV we found a reduction in rabphilin 3a, a protein involved in vesicle docking and recycling. These losses appear to be specific and not due to a general loss of synapses in the HD cortex. Thus, levels of synaptobrevin II, syntaxin 1, rab3a or synaptophysin are unaltered in the same patient samples. SNAP 25 and rabphilin 3a are crucial for neurotransmitter release. Therefore, we suggest that a deficient pre-synaptic transmitter release may underlie some of the symptoms of HD.},
  author       = {Smith, Ruben and Klein, Pontus and Koc-Schmitz, Yeliz and Waldvogel, Henry J and Faull, Richard L M and Brundin, Patrik and Plomann, Markus and Li, Jia-Yi},
  issn         = {1471-4159},
  language     = {eng},
  number       = {1},
  pages        = {115--123},
  publisher    = {Wiley-Blackwell},
  series       = {Journal of Neurochemistry},
  title        = {Loss of SNAP-25 and rabphilin 3a in sensory-motor cortex in Huntington's disease.},
  url          = {http://dx.doi.org/10.1111/j.1471-4159.2007.04703.x},
  volume       = {103},
  year         = {2007},
}