Source:http://linkedlifedata.com/resource/pubmed/id/11555629
Switch to
| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions | |
| pubmed:issue |
18
|
| pubmed:dateCreated |
2001-9-13
|
| pubmed:abstractText |
Amyotrophic lateral sclerosis (ALS) is mainly a sporadic neurodegenerative disorder characterized by loss of cortical and spinal motoneurons. Some familial ALS cases (FALS) have been linked to dominant mutations in the gene encoding Cu/Zn superoxide dismutase (SOD1). Transgenic mice overexpressing a mutated form of human SOD1 with a Gly93Ala substitution develop progressive muscle wasting and paralysis as a result of spinal motoneuron loss and die at 5 to 6 months. We investigated the effects of neurotrophic factor gene delivery in this FALS model. Intramuscular injection of an adenoviral vector encoding cardiotrophin-1 (CT-1) in SOD1G93A newborn mice resulted in systemic delivery of CT-1, supplying motoneurons with a continuous source of trophic factor. CT-1 delayed the onset of motor impairment as assessed in the rotarod test. Axonal degeneration was slowed and skeletal muscle atrophy was largely reduced by CT-1 treatment. By monitoring the amplitude of the evoked motor response, we showed that the time-course of motor impairment was significantly decreased by CT-1 treatment. Thus, adenovirus-mediated gene transfer of neurotrophic factors might delay neurogenic muscular atrophy and progressive neuromuscular deficiency in ALS patients.
|
| pubmed:language |
eng
|
| pubmed:journal | |
| pubmed:citationSubset |
IM
|
| pubmed:chemical | |
| pubmed:status |
MEDLINE
|
| pubmed:month |
Sep
|
| pubmed:issn |
0964-6906
|
| pubmed:author | |
| pubmed:issnType |
Print
|
| pubmed:day |
1
|
| pubmed:volume |
10
|
| pubmed:owner |
NLM
|
| pubmed:authorsComplete |
Y
|
| pubmed:pagination |
1925-33
|
| pubmed:dateRevised |
2006-11-15
|
| pubmed:meshHeading |
pubmed-meshheading:11555629-Adenoviridae,
pubmed-meshheading:11555629-Amyotrophic Lateral Sclerosis,
pubmed-meshheading:11555629-Animals,
pubmed-meshheading:11555629-Animals, Newborn,
pubmed-meshheading:11555629-Atrophy,
pubmed-meshheading:11555629-Behavior, Animal,
pubmed-meshheading:11555629-Body Weight,
pubmed-meshheading:11555629-Cytokines,
pubmed-meshheading:11555629-Female,
pubmed-meshheading:11555629-Gene Expression,
pubmed-meshheading:11555629-Gene Therapy,
pubmed-meshheading:11555629-Gene Transfer Techniques,
pubmed-meshheading:11555629-Genetic Vectors,
pubmed-meshheading:11555629-Humans,
pubmed-meshheading:11555629-Injections, Intramuscular,
pubmed-meshheading:11555629-Male,
pubmed-meshheading:11555629-Mice,
pubmed-meshheading:11555629-Mice, Inbred Strains,
pubmed-meshheading:11555629-Mice, Transgenic,
pubmed-meshheading:11555629-Muscle, Skeletal,
pubmed-meshheading:11555629-Mutation,
pubmed-meshheading:11555629-Nerve Degeneration,
pubmed-meshheading:11555629-Neuromuscular Diseases,
pubmed-meshheading:11555629-Neuromuscular Junction,
pubmed-meshheading:11555629-Phrenic Nerve,
pubmed-meshheading:11555629-Superoxide Dismutase,
pubmed-meshheading:11555629-Survival Analysis,
pubmed-meshheading:11555629-Time Factors
|
| pubmed:year |
2001
|
| pubmed:articleTitle |
Protective effects of cardiotrophin-1 adenoviral gene transfer on neuromuscular degeneration in transgenic ALS mice.
|
| pubmed:affiliation |
Département de Génétique, Institut Cochin de Génétique Moléculaire, 24, rue du Fg Saint Jacques, 75014 Paris, France.
|
| pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
|