Neural acid extraneural expression of the neuronal ceroid lipofuscinoses genes CLN1, CLN2, and CLN3: Functional implications for CLN3

The neuronal ceroid lipofuscinoses (NCLs) are the most common neurodegenera tive disorders of childhood. We have examined mRNA levels of the CLN1, CLN2 , and CLN3 genes, which are associated with the infantile, late infantile, and juvenile forms of NCL in 64 different human tissues, and have grouped t he results into gastrointestinal tract, central nervous system, glandular/s ecretory, muscle, and carcinoma tissue types, mRNA levels for CLN3 are high est in gastrointestinal tissue and are also high in glandular/secretory tis sue, whereas mRNA levels for CLN1 and CLN2 do not appear to be preferential ly elevated in any tissue type. The significance of extraneural expression of CLN3 is reviewed in the context of the function of the protein. (C) 2000 Academic Press.