Sensory and Sympathetic Denervation in the Foot Pads of Mice with a Defect in Bullous Pemphigoid Antigen 1 (BPAG1) Gene

Abstract

Dystonia musculorum (dt) mice are autosomal recessive hereditary mutants with a defect in the bullous pemphigoid antigen 1 (BPAG1) gene. The neural isoform of BPAG1 (BPAG1) is expressed in various neurons, including those in the central and peripheral nervous systems of wild-type mice. Most previous studies on BPAG1- deficient mice have focused on sensory neuronal degeneration; limited investigation of sympathetic innervations has been conducted. To investigate the degeneration of peripheral nerves, patterns of nerve innervation in the skin of dt mice were observed and compared with those in wild-type mice. Nerve innervation was assayed using a general neuronal marker protein gene product 9.5 (PGP 9.5) and the sympathetic neuronal marker tyrosine hydroxylase (TH) with immunoreactivity. Quantitative results indicate that the deficiency in the cytoskeletal linker BPAG1 is responsible for the significant down-regulation in peripheral sensory nerves and sympathetic innervation of the sweat gland in the skin of dt mice foot pads.