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Laminin {alpha}1 chain improves laminin {alpha}2 chain deficient peripheral neuropathy.

Gawlik, Kinga LU ; Li, Jia-Yi LU ; Petersén, Åsa LU and Durbeej-Hjalt, Madeleine LU (2006) In Human Molecular Genetics 15(18). p.2690-2700
Abstract
Absence of laminin alpha2 chain leads to a severe form of congenital muscular dystrophy (MDC1A) associated with peripheral neuropathy. Hence, future therapies should be aimed at alleviating both muscle and neurological dysfunctions. Pre-clinical studies in animal models have mainly focused on ameliorating the muscle phenotype. Here we show that transgenic expression of laminin alpha1 chain in muscles and the peripheral nervous system of laminin alpha2 chain deficient mice reduced muscular dystrophy and largely corrected the peripheral nerve defects. The presence of laminin alpha1 chain in the peripheral nervous system resulted in near-normal myelination, restored Schwann cell basement membranes and improved rotarod performance. In summary,... (More)
Absence of laminin alpha2 chain leads to a severe form of congenital muscular dystrophy (MDC1A) associated with peripheral neuropathy. Hence, future therapies should be aimed at alleviating both muscle and neurological dysfunctions. Pre-clinical studies in animal models have mainly focused on ameliorating the muscle phenotype. Here we show that transgenic expression of laminin alpha1 chain in muscles and the peripheral nervous system of laminin alpha2 chain deficient mice reduced muscular dystrophy and largely corrected the peripheral nerve defects. The presence of laminin alpha1 chain in the peripheral nervous system resulted in near-normal myelination, restored Schwann cell basement membranes and improved rotarod performance. In summary, we postulate that laminin alpha1 chain is an excellent substitute for laminin alpha2 chain in multiple tissues and suggest that treatment with laminin alpha1 chain may be beneficial for MDC1A in humans. (Less)
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; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Human Molecular Genetics
volume
15
issue
18
pages
2690 - 2700
publisher
Oxford University Press
external identifiers
  • wos:000240590400004
  • pmid:16893907
  • scopus:33748750613
  • pmid:16893907
ISSN
0964-6906
DOI
10.1093/hmg/ddl201
language
English
LU publication?
yes
additional info
The information about affiliations in this record was updated in December 2015. The record was previously connected to the following departments: Neuronal Survival (013212041), Muscle biology (013212015)
id
088fc5c3-1076-45e7-8dbc-5c13fb5304ba (old id 160231)
alternative location
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16893907&dopt=Abstract
date added to LUP
2016-04-01 12:22:49
date last changed
2020-12-08 04:59:00
@article{088fc5c3-1076-45e7-8dbc-5c13fb5304ba,
  abstract     = {Absence of laminin alpha2 chain leads to a severe form of congenital muscular dystrophy (MDC1A) associated with peripheral neuropathy. Hence, future therapies should be aimed at alleviating both muscle and neurological dysfunctions. Pre-clinical studies in animal models have mainly focused on ameliorating the muscle phenotype. Here we show that transgenic expression of laminin alpha1 chain in muscles and the peripheral nervous system of laminin alpha2 chain deficient mice reduced muscular dystrophy and largely corrected the peripheral nerve defects. The presence of laminin alpha1 chain in the peripheral nervous system resulted in near-normal myelination, restored Schwann cell basement membranes and improved rotarod performance. In summary, we postulate that laminin alpha1 chain is an excellent substitute for laminin alpha2 chain in multiple tissues and suggest that treatment with laminin alpha1 chain may be beneficial for MDC1A in humans.},
  author       = {Gawlik, Kinga and Li, Jia-Yi and Petersén, Åsa and Durbeej-Hjalt, Madeleine},
  issn         = {0964-6906},
  language     = {eng},
  number       = {18},
  pages        = {2690--2700},
  publisher    = {Oxford University Press},
  series       = {Human Molecular Genetics},
  title        = {Laminin {alpha}1 chain improves laminin {alpha}2 chain deficient peripheral neuropathy.},
  url          = {http://dx.doi.org/10.1093/hmg/ddl201},
  doi          = {10.1093/hmg/ddl201},
  volume       = {15},
  year         = {2006},
}