Reis, S.

inclusion protein mouse fxr 1 þ brain fxr 1p muscle level antibody intranuclear inclusions tissue fxr 1 ko premutation study neuron syndrome expanded-repeat intranuclear analysis genet model presence expression genetic number vinculin section expanded-repeat mice x syndrome nuclei fmr 1 gene figure wt littermates littermate disease fxr 1 cerebellar nucleus parafascicular thalamic nucleus instability hagerman retardation neonate addition retardation protein carrier proteasome dystrophin result premutation carriers ubiquitin-positive intranuclear inclusions fmr 1 mrna ubiquitin-positive tremor cortex expanded-repeat mouse phenotype a-actinin brain tissue fmrp levels warren tremor /ataxia syndrome ubiquitin rabbit function absence patient fxr 2p clone region costameric expansion costamere speci actin tassone lox p sites mouse model wt mice cassette