Mouse anti-ATPase p97 monoclonal antibody for WB. Suitable for use in Western blot (1:1,000 – 1:2,000). Not for use in immunofluorescence and immunoprecipitation. Each laboratory should determine an optimum working titer for use in its particular application. Other applications have not been tested but use in such assays should not necessarily be excluded.Defects in VCP arethe cause of inclusion body myopathy with early-onset Paget disease andfrontotemporal dementia (IBMPFD) [MIM:167320]; also known as musculardystrophy, limb-girdle, with Paget disease of bone or pagetoid amyotrophiclateral sclerosis or pagetoid neuroskeletal syndrome or lower motor neurondegeneration with Paget-like bone disease. IBMPFD features adult-onsetproximal and distal muscle weakness (clinically resembling limb girdlemuscular dystrophy), early-onset Paget disease of bone in most cases andpremature frontotemporal dementia.
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