α-Internexin and Peripherin: Expression, Assembly, Functions, and Roles in Disease

Methods in Enzymology
Jian Zhao, Ronald K H Liem

Abstract

α-Internexin and peripherin are neuronal-specific intermediate filament (IF) proteins. α-Internexin is a type IV IF protein like the neurofilament triplet proteins (NFTPs, which include neurofilament light chain, neurofilament medium chain, and neurofilament high chain) that are generally considered to be the primary components of the neuronal IFs. However, α-internexin is often expressed together with the NFTPs and has been proposed as the fourth subunit of the neurofilaments in the central nervous system. α-Internexin is also expressed earlier in the development than the NFTPs and is a maker for neuronal IF inclusion disease. α-Internexin can self-polymerize in vitro and in transfected cells and it is present in the absence of the NFTP in development and in granule cells in the cerebellum. In contrast, peripherin is a type III IF protein. Like α-internexin, peripherin is specific to the nervous system, but it is expressed predominantly in the peripheral nervous system (PNS). Peripherin can also self-assemble both in vitro and in transfected cells. It is as abundant as the NFTPs in the sciatic nerve and can be considered a fourth subunit of the neurofilaments in the PNS. Peripherin has multiple isoforms that arise from intron ...Continue Reading

Citations

Jun 22, 2017·Journal of Experimental Neuroscience·Koshiro KamiyaMasao Koda
Dec 3, 2017·Cold Spring Harbor Perspectives in Biology·Elly M Hol, Yassemi Capetanaki
Jan 24, 2020·Cytoskeleton·Christopher J Bott, Bettina Winckler
May 4, 2017·Molecular Neurobiology·Moritz OberstadtMax Holzer
Nov 15, 2018·Frontiers in Cellular Neuroscience·Anna M ZeitlbergerPaola Giunti
Mar 2, 2021·Journal of Agricultural and Food Chemistry·Rongmao HuaQingwang Li
Mar 17, 2021·Translational Stroke Research·Fani Pujol-CalderónLars E Rosengren
Jul 16, 2021·Chemico-biological Interactions·David Hernandez-Toledano, Libia Vega
Jul 16, 2021·Annals of Clinical and Translational Neurology·Daniele SabbatiniGianni Sorarù

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