Article
Overcoming functional redundancy to elicit pachyonychia congenita-like nail lesions in transgenic mice.
Molecular and cellular biology - 1 Jan 2005
Wong Pauline, Domergue Renee, Coulombe Pierre A
Abstract excerpt
Mutations affecting the coding sequence of intermediate filament (IF) proteins account for >30 disorders, including numerous skin bullous diseases, myopathies, neuropathies, and even progeria. The manipulation of IF genes in mice has been widely successful for modeling key features of such clinically distinct disorders. A notable exception is pachyonychia congenita (PC), a disorder in which the nail and other...
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