C. Chavany et al., TRANSGENIC MICE FOR INTERLEUKIN-3 DEVELOP MOTOR-NEURON DEGENERATION ASSOCIATED WITH AUTOIMMUNE REACTION AGAINST SPINAL-CORD MOTOR-NEURONS, Proceedings of the National Academy of Sciences of the United Statesof America, 95(19), 1998, pp. 11354-11359
Interleukin 3 (IL-3) stimulates the proliferation and differentiation
of various haematopoietic progenitor cells. Recently, IL-3 and other c
ytokines were reported to exert a neurotrophic activity and to be asso
ciated with neurological disorders, suggesting their complex role in t
he central nervous system. We now show that overexpression of IL-3 in
transgenic mice causes a motor neuron disease with several features of
amyotrophic lateral sclerosis and progressive muscular atrophy. These
animals exhibit hind limb paralysis at 7 months of age, associated wi
th dendritic and axonal degeneration, loss of motor neurons in the spi
nal cord, and autoimmune reaction against these cells. We examined the
effect of IL-3 on embryonic motor neurons survival in mixed spinal co
rd cultures. Our results suggest that motor neuronal degeneration is n
ot directly triggered by the high level of expression of IL-3.