1. S100A6, a calcium- and zinc-binding protein, is overexpressed in SOD1 mutant mice, a model for amyotrophic lateral sclerosis
- Author
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Daphné Hoyaux, Robert Kiss, Claus W. Heizmann, Jules Alao, Julia Fuchs, Detlev Frermann, Bernhard U. Keller, Roland Pochet, Centre interdisciplinaire de recherche en biologie (CIRB), Labex MemoLife, École normale supérieure - Paris (ENS Paris), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Collège de France (CdF (institution))-Ecole Superieure de Physique et de Chimie Industrielles de la Ville de Paris (ESPCI Paris), Université Paris sciences et lettres (PSL)-École normale supérieure - Paris (ENS Paris), and Université Paris sciences et lettres (PSL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)
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Male ,[SDV]Life Sciences [q-bio] ,Cell Cycle Proteins ,MESH: Calcium-Binding Proteins ,MESH: Zinc ,S100 Calcium Binding Protein A6 ,MESH: Spinal Cord ,Mice ,0302 clinical medicine ,Superoxide Dismutase-1 ,Calcium-binding protein ,MESH: Glial Fibrillary Acidic Protein ,MESH: Animals ,Amyotrophic lateral sclerosis ,S100A6 ,MESH: Amyotrophic Lateral Sclerosis ,MESH: Superoxide Dismutase ,MESH: Superoxide Dismutase-1 ,0303 health sciences ,Neurodegeneration ,S100 Proteins ,Anatomy ,Immunohistochemistry ,3. Good health ,Motoneuron ,Zinc ,Spinal Cord ,MESH: S100 Proteins ,Genetically modified mouse ,Hypoglossal nucleus ,MESH: Mice, Transgenic ,Calcium binding protein ,Calcium buffering ,SOD1 ,MESH: Carrier Proteins ,Mice, Transgenic ,Biology ,Superoxide dismutase ,03 medical and health sciences ,MESH: Cell Cycle Proteins ,Glial Fibrillary Acidic Protein ,medicine ,Animals ,Humans ,MESH: Mice ,Molecular Biology ,030304 developmental biology ,MESH: Humans ,Superoxide Dismutase ,Amyotrophic Lateral Sclerosis ,Calcium-Binding Proteins ,MESH: S100 Calcium Binding Protein A6 ,MESH: Immunohistochemistry ,Cell Biology ,medicine.disease ,Molecular biology ,MESH: Male ,MESH: Astrocytes ,Disease Models, Animal ,nervous system ,Reactive astrocyte ,Astrocytes ,MESH: Brain Stem ,biology.protein ,MESH: Disease Models, Animal ,Carrier Proteins ,030217 neurology & neurosurgery ,Brain Stem - Abstract
International audience; Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterised by selective degeneration of motoneurones. Familial ALS is an age-dependent autosomal dominant disorder in which mutations in the homodimeric enzyme Cu/Zn superoxide dismutase 1 (SOD1) is linked to the disease. An animal model for this disease is a transgenic mouse expressing the mutated human SOD1(G93A) gene. Recent electrophysiological data emphasised that the striking selective vulnerability of motoneurones might be due to their differential calcium buffering capacities. Therefore we have investigated, using immunohistochemistry, the expression of different calcium binding proteins in brainstem and spinal cord from normal and SOD1 mutated mice. Among the 13 calcium-binding proteins screened, only one, S100A6, a homodimeric calcium-binding protein able to bind four Zn(2+), appeared to be highly expressed in the SOD1 mutated mice. In brainstem, reactive astrocytes, but not motoneurones, from several regions, including nerve 12 root, were highly S100A6-positive. Hypoglossal nucleus was negative for S100A6. In dorsal root, reactive astrocytes from both white matter and anterior horn were highly reactive. If overexpression of S100A6 is specific for ALS, it will be a valuable diagnostic marker for this disease.
- Published
- 2000
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