Recombinant Human Peripherin (PRPH)

Uniprot Id

P41219

Target Species

Human

Target Name

PRPH

Target Full Name

Peripherin

Target Function

Class-III neuronal intermediate filament protein. May form an independent structural network without the involvement of other neurofilaments or may cooperate with the neuronal intermediate filament proteins NEFL, NEFH, NEFM and INA to form a filamentous network. Assembly of the neuronal intermediate filaments may be regulated by RAB7A. Plays a role in the development of unmyelinated sensory neurons. May be involved in axon elongation and axon regeneration after injury. Inhibits neurite extension in type II spiral ganglion neurons in the cochlea.

Target Subcellular Location

Cytoplasm, cytoskeleton. Cell projection, axon. Perikaryon.

Target Protein Families

Intermediate filament family

Target Tissue Specificity

Expressed in the neurons of the outer hair cells in the organ of Corti and to a lesser extent in type I spiral ganglion cells.

Target Synonyms

NEF 4; NEF4; Neurofilament 4 (57kD) ; Neurofilament 4; Perf; PERI_HUMAN; Peripherin; PRPH 1; prph; PRPH1

Target Background

This gene encodes a cytoskeletal protein found in neurons of the peripheral nervous system. The encoded protein is a type III intermediate filament protein with homology to other cytoskeletal proteins such as desmin, and is a different protein that the peripherin found in photoreceptors. Mutations in this gene have been associated with susceptibility to amyotrophic lateral sclerosis.