A mutation in the microtubule-associated protein tau in pallido-nigroluysian degeneration

We detected a missense mutation in exon 10 of tau that causes a substitutio n at codon 279 (N279K)in a Japanese patient with a familial background of p arkinsonism and dementia originally described as pallido-nigro-luysian dege neration. This mutation is the same as one seen in a Caucasian family with pallido-ponto-nigral degeneration. The similarities between these two famil ies suggest a common genetic mechanism that may account for the peculiar di stribution of neuroglial degeneration with tauopathy.