Peripherin

Arikawa, K., Molday, L. L., Molday, R. S. and Williams, D. S. Localization of peripherine/rds in the disk membranes of cone and rod photoreceptors relationship to disk membrane morphogenesis and retinal degeneration. /. Cell Biol. 116 659-667,1992. 

Desmin, Vimentin, Glial Fibrillary Acidic Protein, and Peripherin (Type III)... 

Desmin, Vimentin, Glial Fibrillary Acidic Protein, and Peripherin (Type III), Neurofilaments and a-Intemexin (Type TV), Lamins (Type V), and Nestin (Type VI)... 

The role of the IF, and particularly the keratin filament system, in resisting the forces of mechanical stress has been well established. However, IFs also play a role in countering metabolic stress. Perhaps the best example is the cytoprotective role played by the simple epithelial keratins, K8/18. However, vimentin, desmin, peripherin, GFAP, the lens proteins phakinin, and filensin and other keratins have also been shown to associate with members of the small heat shock protein (HSP) family, including HSP27 and aB-crystallin (reviewed in Coulombe and Wong, 2004 Marceau et al., 2001 Nicholl and Quinlan, 1994). 

Vimentin is not the only IF type that is moved in this manner. Neural IFs, including both the type III peripherin proteins and type IV neural IF proteins, have also been demonstrated to be moved by molecular motors. In the case of peripherin, particles and squiggles were observed to translocate rapidly within PCI 2 cell bodies, neurites, and growth cones. The movements were bidirectional and dependent on microtubules, kinesin, and cytoplasmic dynein. The authors suggest that peripherin particles are... 

Leung, C. L., Sun, D., and Liem, R. K. (1999). The intermediate filament protein peripherin is the specific interaction partner of mouse BPAGl-n (dystonin) in neurons./. Cell Biol. 144, 435-446. 

Beaulieu JM, Nguyen MD, Julien JP (1999) Late onset of motor neurons in mice oveiexpressing wild-type peripherin. J Cell Biol 147 531-544... 

Neurofilament and peripherin mutations were reported in rare forms of ALS (Gros-Louis et al., 2004 Leung et at., 2004), leading researchers to develop animal models bearing these mutations (Millecamps et al., 2006). Although these mice developed no evident MND, some exhibited moderate sensorimotor and spatial deficits probably due to the observed reduction in conduction velocity. 

Gros-Louis F, Lativiere R, Gowing G, Laurent S, Camu W, Bouchard JP, Meimnger V, Rouleau GA, JuUen JP (2004) A frameshift deletion in peripherin gene associated with amyotrophic lateral sclerosis. J Biol Chem 279 45951-45956... 

Millecamps S, Robertson J, Laiiviere R, MaUet J, Juhen JP (2006) Defective axonal transport of neurofilament proteins in neurons overexpressing peripherin. J Neurochem 98 926-938... 

Poetsch, A., Molday, L.L. and Molday, R.S. (2001) The cGMP-gated channel and related glutamic acid-rich proteins interact with peripherin-2 at the rim region of rod photoreceptor disc membranes. J. Biol. 

Five classes of intermediate filament proteins have been described, generally referred to as types I through V. Types I and II are the acidic and basic polypeptides, respectively, which comprise the keratins and cytokeratins, a family of heteropolymers that are abundant in epithelia. Type III consist of vimentin, desmin, glial fibrillary acidic protein, and peripherin. Of these, desmin has specific importance... 

Keen TJ, Inglehearn CF. 1996. Mutations and polymorphisms in the human peripherin-RDS gene and their involvement in inherited retinal degeneration. Hum Mutat 8 ... 

Bok D, Yasumura D, Matthes M, et al. Effects of adeno-associated virus-vectored ciliary neurotrophic factor on retinal structure and function in mice with a P216L rds/ peripherin mutation. Exp Eye Res 2002 74 719-735. 

Wong, J. and Oblinger, M.M. (1990) Differential regulation of peripherin and neurofilament gene expression in regenerating rat DRG neurons. J. Neurosci. Res. 27 332-341. 

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