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A Dystrophin‐Immunoreactive Protein in Mammalian Brain
Author(s) -
Clerk Angela,
Muntoni Francesco,
Strong Peter N.
Publication year - 1993
Publication title -
journal of neurochemistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.75
H-Index - 229
eISSN - 1471-4159
pISSN - 0022-3042
DOI - 10.1111/j.1471-4159.1993.tb03169.x
Subject(s) - dystrophin , spectrin , utrophin , biology , mdx mouse , gene , gene product , mutant , microbiology and biotechnology , genetics , gene expression , cytoskeleton , cell
Dystrophin is expressed only in muscle and brain, but is absent from all tissues of the adult mdx mouse, a mutant with a single base substitution in the dystrophin gene. The brains of both normal and mdx mice contain a protein of ∼230 kDa that is recognised by anti‐dystrophin antibodies raised to the N‐terminal region of the rod‐like domain. Although the N‐terminal and central rod regions of dystrophin share structural homologies with spectrin, the 230‐kDa protein represents neither of the presently described forms of brain spectrin by a variety of criteria (molecular weight, cerebellar localisation, and developmental regulation) and is distinct from the product of the dystrophin gene. Studies of mdx and normal mouse brain show different postnatal developmental regulation of the 230‐kDa dystrophin‐immunoreactive protein.