title: Modulation of HSP25 expression during anterior horn motor neuron degeneration in the paralysé mouse mutant.
abstract: The paralysé spontaneous mutation in mice involves degeneration and death of anterior horn motor neurons. Mutant mice are not viable past postnatal day 16. At present, the mechanisms involved in motor neuron death are unknown. Here, we investigate the expression of the small heat shock protein Hsp25, in the spinal cord of paralysé at two different stages during postnatal development, i.e., day 11 and day 14. Western blot analysis reveals that the level of Hsp25 was strikingly different in paralysé as compared to control littermates. Hsp25 expression level in paralysé at day 11 was much lower than in control mice. At day 14, an opposite pattern was observed. Such pattern seems to be restricted to spinal cord, since level of Hsp25 in other tissues (lung, brain, liver, and heart) was quite similar. Immunofluorescence examination of the lumbar spinal cord sections reveals that in control mice, Hsp25 was expressed at high level in motor neurons located in the ventral horn at both day 11 and day 14. By contrast, in paralysé mice, Hsp25 staining within the motor neurons was barely detectable except as a spot in the nucleolus (day 11). At the end stage of the disease (day 14), not only was Hsp25 staining even less intense in motor neurons, but also a strong Hsp25 staining was observed in reactive astrocytes within the gray matter. Taken together, these data suggest that Hsp25 expression is differently modulated in neuronal and glial cells during neurodegenerative processes leading to motor neuron death.
authors.affiliation: CNRS UMR 8505/ENS-LSH, Le Parc, 92211 Saint Cloud Cedex, France.
authors.forename: I
authors.initials: I
authors.lastname: Pieri
authors.forename: C
authors.initials: C
authors.lastname: Cifuentes-Diaz
authors.forename: J P
authors.initials: JP
authors.lastname: Oudinet
authors.forename: B
authors.initials: B
authors.lastname: Blondet
authors.forename: F
authors.initials: F
authors.lastname: Rieger
authors.forename: S
authors.initials: S
authors.lastname: Gonin
authors.forename: A P
authors.initials: AP
authors.lastname: Arrigo
authors.forename: Y
authors.initials: Y
authors.lastname: Thomas
mesh_terms: D000818:Animals; D000870:Anterior Horn Cells / Q000378:metabolism* / Q000473:pathology; D017209:Apoptosis; D001253:Astrocytes / Q000473:pathology; D015153:Blotting, Western; D004195:Disease Models, Animal; D005455:Fluorescent Antibody Technique; D020869:Gene Expression Profiling; D005786:Gene Expression Regulation; D005838:Genotype; D005904:Glial Fibrillary Acidic Protein / Q000032:analysis; D006360:Heat-Shock Proteins; D051379:Mice; D008809:Mice, Inbred C3H; D008818:Mice, Neurologic Mutants; D018832:Molecular Chaperones; D016472:Motor Neuron Disease / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D009363:Neoplasm Proteins / Q000096:biosynthesis* / Q000235:genetics; D009410:Nerve Degeneration; D009419:Nerve Tissue Proteins / Q000096:biosynthesis* / Q000235:genetics
chemical_list: D005904:Glial Fibrillary Acidic Protein; D006360:Heat-Shock Proteins; C486113:Hsbp1 protein, mouse; D018832:Molecular Chaperones; D009363:Neoplasm Proteins; D009419:Nerve Tissue Proteins