[Role of caspases in neural degeneration in amyotrophic lateral sclerosis].
Animal model of the motor neuron disease amyotrophic lateral sclerosis (ALS), has implicated apoptotis in the neuronal degeneration evident in this disease. Mice showing ALS-like symptoms, as well as several human families with ALS, have a missense mutation in the gene coding for DOD-1. A defective enzyme enhances neuronal cell death by activating interleukin converting enzyme (ICE), an initia of apoptosis. The role of caspase-1 and caspase-3 in the apoptotic process is apparent and the use of the caspase inhibitor zVAD-fink demonstrated some promise in slowing neural death by apoptosis and prolonging life in mice with ALS-like symptoms. We discuss the possible mechanisms of caspase enzymes cascade activation and their central role in apoptosis and possibly in neural death.
Sela
B
B
Department of Pathological Chemistry, Sheba Medical Center, Tel Hashomer.
D020170:Caspase 1 / Q000378:metabolism; D053143:Caspase 2; D020169:Caspases / Q000378:metabolism*; D006801:Humans; D016472:Motor Neuron Disease / Q000201:enzymology / Q000503:physiopathology*; D009410:Nerve Degeneration / Q000503:physiopathology*; D015398:Signal Transduction
D053143:Caspase 2; D020169:Caspases; D020170:Caspase 1