AMPA Receptor & ALS

By Philip Van Damme
September 2004
Leuven University Press
ISBN: 9058673901
112 pages, Illustrated, 6 " x 9 "
$57.50 Paper Original


This is a Ph.D. dissertation. Amyotrophic lateral sclerosis (ALS), first described by Jean-Martin Charcot in 1869, is a devastating neurodegenerative disease characterized by a progressive loss of upper motor neurons in the motor cortex and lower motor neurons in the brainstem and spinal cord. ALS typically affects adults in mid-life, with an incidence of 1 to 2/100,000. The majority of ALS patients has no affected family members and is considered to have sporadic ALS.

Familial ALS occurs in 5 to 10% of cases and predominantly has an autosomal dominant inheritance. In 20% of familial cases (which represents only 1 to 2% of all cases) mutations in the superoxide dismutase-1 (SOD1) gene on chromosome 21q can be identified. Recently, a very rare recessive form of juvenile ALS has been found to be caused by mutations in alsin, which codes for a protein containing guanine exchange factor domains. Several other loci for classical ALS have been found, but additional disease-causing mutations still await identification. The etiology of sporadic ALS remains obscure.

Contents include: Introduction, Materials and methods, Contribution of AMPA receptor-mediated excitotoxicity to motor neuron degeneration in a mouse model of ALS, Mechanism of AMPA receptor-mediated excitotoxicity in cultured motor neurons: ionic dependence, Role of GluR2 in the selective vulnerability of motor neurons to AMPA receptor-mediated excitotoxicity, Role of astrocytes in GluR2 expression in motor neurons, General discussion and perspectives.

Neurology
Acta Biomedica Lovaniensia No. 316

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