Peripherin [A3]

Peripherin is a type III intermediate filament protein that is expressed in peripheral and some central nervous system (CNS) neurons, which extends to many tissues throughout the body including the salivary gland, small intestine, prostate, stomach, and colon. Peripherin activation is known to be induced by leukemia inhibitory factor (LIF). LIF activates Peripherin by inducing members of Stat transcription factor family to bind to a specific promoter element in the Peripherin gene. IL-6 is also known to induce Peripherin expression. Although it is not essential for neurite formation, Peripherin is necessary for cellular intermediate filament network formation. Peripherin, unlike most intermediate filament proteins, has been reported to be modified by tyrosine phosphorylation. The use of anti-peripherin in tracking the reduction or loss of ganglion cells in the submucosal and myenteric layers of the colon wall can act as a valuable tool in identifying patients suspected of recto-sigmoid Hirschsprung disease and other forms of colonic aganglionosis.