Decreased insulin-like growth factor I-mediated protein tyrosine phosphorylation in human olivopontocerebellar atrophy and lurcher mutant mouse
| Autor: | Robert D. Currier, Jonathan D. Fratkin, P. Joshi, Parminder J. S. Vig, Durisala Desaiah, S. H. Subramony |
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| Rok vydání: | 1994 |
| Předmět: |
Male
medicine.medical_specialty Cerebellum Blotting Western Molecular Sequence Data Lurcher Biology Receptor IGF Type 1 Iodine Radioisotopes Mice Mice Neurologic Mutants chemistry.chemical_compound Olivopontocerebellar atrophy Internal medicine medicine Animals Amino Acid Sequence Insulin-Like Growth Factor I Phosphorylation Cell Membrane Autophosphorylation Tyrosine phosphorylation Protein-Tyrosine Kinases medicine.disease Endocrinology medicine.anatomical_structure Neurology chemistry Cerebellar cortex Nerve Degeneration Olivopontocerebellar Atrophies Autoradiography Female Neurology (clinical) Peptides Tyrosine kinase Signal Transduction |
| Zdroj: | Journal of the Neurological Sciences. 124:38-44 |
| ISSN: | 0022-510X |
| Popis: | We examined insulin-like growth factor I (IGF-I)-dependent phosphorylation and protein tyrosine kinase (PTK) activity in cerebellar cortex of normal humans, patients with olivopontocerebellar atrophy (OPCA) ("C" kindred) and in lurcher mutant mouse, a suggested animal model for OPCA. PTK activity and IGF-I-dependent protein tyrosine phosphorylation was significantly reduced in cerebellar cortex of human OPCA patients as compared to the normal controls. Immunoblot analysis also demonstrated a decrease in cerebellar 80 kDa phosphotyrosine protein in these patients. By autoradiography, IGF-I receptors were localized in the molecular layer of 30-day-old control and lurcher mutant mice cerebella. However, the lurcher mutant mice showed a decrease in [ 125 I]-IGF-I binding in the molecular layer as compared to the littermate controls. The IGF-I receptor autophosphorylation was also markedly reduced in 15-day- and 22-day-old lurcher cerebella. These results suggest that the process of cerebellar degeneration in human OPCA and lurcher mutant mouse may be associated with altered IGF-I receptor binding and protein tyrosine phosphorylation. |
| Databáze: | OpenAIRE |
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