ACETYLCHOLINE ESTERASE AND PERIPHERIN MESSENGER-RNA LEVEL DECREASE INWOBBLER MOUSE

HOMOZYGOTE wobbler mice develop motoneurone degeneration. Throughout d evelopment the expression of choline acetyltransferase, of trkC recept or and F3 adhesion molecule genes is similar in wobbler and wildtype s pinal cord. Acetylcholinesterase mRNA level instead is decreased to ab out 50% with respect to wild-type values in one forth of P5 and P10 wo bbler progeny, putative wr/wr individuals; at P21 its expression is eq ually highly reduced in known homozygotes and it is reduced to 35% of normal values in about one half of the progeny, putative heterozygotes . Thus, similarly to medium neurofilament gene over-expression, reduce d acetylcholinesterase gene expression is an early molecular marker fo r the wobbler mutation before onset of the illness.