The muscle protein dysferlin accumulates in the Alzheimer brain

摘要: Dysferlin is a transmembrane protein that highly expressed in muscle. mutations cause limb-girdle dystrophy type 2B, Miyoshi myopathy and distal anterior compartment myopathy. has also been described neural tissue. We studied dysferlin distribution the brains of patients with Alzheimer disease (AD) controls. Twelve brains, staged using Clinical Dementia Rating were examined: 9 AD cases (mean age: 85.9 years mean duration: 8.9 years), 3 age-matched controls 87.5 years). cytoplasmic pyramidal neurons normal brains. In addition, there dysferlin-positive dystrophic neurites within Aβ plaques brain, distinct from tau-positive neurites. Western blots total brain (RIPA) sequential extraction buffers (high salt, high salt/Triton X-100, SDS formic acid) increasing strength performed to examine solubility state. RIPA fractions, was seen as 230–272 kDa bands serial extractions, shift soluble phase X-100 more insoluble fraction AD. new accumulates association neuritic plaques. muscle, plays role repair muscle membrane damage. The accumulation may be related inability damage due deposits accumulating brain.