Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease

Article Abstract:

Huntington's disease (HD) and reversal of neuropathology and motor dysfunction in a conditional model of the disease are discussed. Neurodegenerative disorders of this type are characterized by progressive and what is thought to be irreversible clinical and neuropathological symptoms. A tetracycline-regulatable system with mice expressing a mutated huntingtin fragment has been used to set up the conditional HD model. The mice show neuronal inclusions, characteristic neuropathology and progressive motor dysfunction. Blockage of expression in mice with symptoms leads to disappearance of inclusions and amelioration of the behavioral phenotype. Continuous inflow of a mutant protein is needed to maintain inclusions and symptoms. There is thus some possibility that HD could be reversed.

Nervous system, Neurons, Huntington's chorea, Huntington's disease, Nerve degeneration, Tetracycline, Tetracyclines

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Heterozygous germline mutations in the p53 homolog p63 are the cause of EEC syndrome

Article Abstract:

Heterozygous germline mutations in the p53 homolog p63 are discussed and shown to be cause of EEC syndrome, which takes in ecrodactyly, ectrodermal dysplasia and cleft lip with/without cleft palate and may affect the hair, skin, nails and teeth. The region was implicated in the EEC-like disorder limb mammary syndrome (LMS). Analysis of the p63 gene showed heterozygous mutations in nine unrelated EEC families. A molecular explanation for the dominant nature of p63 mutations in EEC syndrome is seen.

United Kingdom, Netherlands, Belgium, Statistical Data Included, Abnormalities, Pediatric diseases, Hand, Chromosome mapping, Homology (Biology), Face, Facies (Medicine), Syndromes in children, Syndromes, Foot, Genitourinary organs, Foot deformities, Urogenital abnormalities

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Measles virus infection in a transgenic model: virus-induced immunosuppression and central nervous system disease

Article Abstract:

An effort to use a CD46 transgenic mouse model to study measles virus (MV) pathogenesis and its effects on the central nervous and immune systems is discussed. Expression of the MV receptor CD46 closely mimicked the location and amount of CD46 found in humans. A small animal model is now available for MV pathogenesis analysis. Lack of such a model has inhibited progress in the study of how MV causes disease and in the development of novel therapies better vaccines.

Physiological aspects, Development and progression, Measles, Central nervous system diseases, Immunosuppression, Measles virus

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