Overview
- Peptide (C)DVEFSFPKFSRLRR, corresponding to amino acid residues 155-170 of rat PRX (Accession Q63425). Intracellular, N-terminus.
Western blot analysis of rat DRG (lanes 1 and 3) and mouse brain (lanes 2 and 4) lysates:1,2. Anti-Periaxin Antibody (#ANR-091), (1:600).
3,4. Anti-Periaxin Antibody, preincubated with Periaxin Blocking Peptide (#BLP-NR091).
Expression of Periaxin in rat peripheral nerveImmunohistochemical staining of free floating frozen rat peripheral nerve sections using Anti-Periaxin Antibody (#ANR-091), (#1:600). A. PRX staining (green) appears in a peripheral nerve (arrow) connecting to the spinal dorsal horn (D horn). Note the absence of staining of spinal myelin column indicating the specificity of the antibody to peripheral myelin. B. A higher magnification of the insert from panel A. C. Segment of peripheral nerve contains PRX-positive fibers. (arrows). In all panels DAPI is used as the counterstain (blue).
- Williams, C. and Brophy, P.J. (2002) J. Anat. 200, 323.
- Han, H. and Kursula, P. (2014) J. Biol. Chem. 289, 14121.
- Shi, Y. et al. (2014) PLoS One. 9, 91953.
Periaxin protein is a membrane-associated protein expressed in myelinating Schwann cells. It is required for the stable maintenance and development of a normal myelin sheath in the peripheral nervous system. It is also a member of cytoskeletal complexes in lens fibers where it plays an important role in maturation, packing, and membrane organization of lens fiber cell1,2.
The periaxin gene (PRX) encodes two protein isoforms, L-periaxin and S-periaxin generated by alternative mRNA splicing. These proteins contain N-terminal PDZ domains (PSD-95/Discs-large/ZO-1) important for organizing protein–protein interactions.
L-periaxin is a component of the dystroglycan–dystrophin-related protein-2 complex (DRP2) linking the Schwann cell cytoskeleton to the extracellular matrix. Its location changes during myelination; at first the protein is localized to the adaxonal plasma membrane and later, in the abaxonal plasma membrane. S-periaxin is distributed in a uniform manner in the cytoplasm and the nucleus of the Schwann cell1-3.
Mutations in the Periaxin gene are known to cause autosomal recessive demyelinating in Type 4F Charcot-Marie-Tooth (CMT4F) and Dejerine-Sottas disease3.
Periaxin knockout mice develop normally myelinated peripheral nervous system at first but develop a late onset demyelinating peripheral neuropathy1.
Anti-Periaxin Antibody (#ANR-091) is a highly specific antibody directed against an epitope of rat PRX. The antibody can be used in western blot and immunohistochemistry applications. It has been designed to recognize PRX from mouse, rat and human samples. The antibody specifically recognizes L-Periaxin. It does not recognize S-Periaxin.
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