Article
Mouse gene knockout models for the CLN2 and CLN3 forms of ceroid lipofuscinosis.
European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society - 1 Jan 2001
Katz M L, Johnson G S
Abstract excerpt
The childhood neuronal ceroid-lipofuscinoses (NCLs) are autosomal-recessively inherited neurodegenerative disorders that result in severe cognitive decline and premature death. The genetic bases for a number of different forms of NCL, including those designated CLN2 and CLN3, have now been determined. However, the mechanisms by which the gene defects cause the disease pathology are not known and no effective...
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