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    Proc Natl Acad Sci U S A. 1998 Dec 22;95(26):15763-8.

    Caspase-1 is activated in neural cells and tissue with amyotrophic lateral sclerosis-associated mutations in copper-zinc superoxide dismutase.

    Pasinelli P, Borchelt DR, Houseweart MK, Cleveland DW, Brown RH Jr.

    Source

    Massachusetts General Hospital-East, Charlestown, MA 02129, USA.

    Erratum in

    • Proc Natl Acad Sci U S A 1999 Mar 16;96(6):3330.

    Abstract

    The mechanism by which mutations in the superoxide dismutase (SOD1) gene cause motor neuron degeneration in familial amyotrophic lateral sclerosis (ALS) is unknown. Recent reports that neuronal death in SOD1-familial ALS is apoptotic have not documented activation of cell death genes. We present evidence that the enzyme caspase-1 is activated in neurons expressing mutant SOD1 protein. Proteolytic processing characteristic of caspase-1 activation is seen both in spinal cords of transgenic ALS mice and neurally differentiated neuroblastoma (line N2a) cells with SOD1 mutations. This activation of caspase-1 is enhanced by oxidative challenge (xanthine/xanthine oxidase), which triggers cleavage and secretion of the interleukin 1beta converting enzyme substrate, pro-interleukin 1beta, and induces apoptosis. This N2a culture system should be an instructive in vitro model for further investigation of the proapoptotic properties of mutant SOD1.

    PMID:
    9861044
    [PubMed - indexed for MEDLINE]
    PMCID:
    PMC28118
    Free PMC Article

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