Progressive and selective degeneration of motoneurons in a mouse model of SMA.
Neuroreport
; 15(2): 275-80, 2004 Feb 09.
Article
in En
| MEDLINE
| ID: mdl-15076752
ABSTRACT
Spinal muscular atrophy (SMA), an autosomal recessive disorder characterized by the degeneration of motoneurons of the spinal cord and brainstem, results from loss-of-function mutations in the survival motor neuron gene (smn). The goal of these experiments was to analyse axons and cell bodies of motoneurons in different regions of the CNS during disease progression in a mouse model of SMA carrying a deletion of the exon 7 directed to neurons. These experiments demonstrate a progressive loss of motor axons and of motoneurons in the CNS. This is the first study that describes a selective neurodegeneration in this line of mice and underlines the importance of exon 7 in some populations of motoneurons for survival in vivo.
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Collection:
01-internacional
Database:
MEDLINE
Main subject:
Spinal Cord
/
Brain Stem
/
Muscular Atrophy, Spinal
/
Motor Neurons
/
Nerve Degeneration
/
Nerve Tissue Proteins
Limits:
Animals
Language:
En
Journal:
Neuroreport
Journal subject:
NEUROLOGIA
Year:
2004
Document type:
Article
Affiliation country:
Switzerland