The Pathological Role of Loss-Function of FUS-NLS Mutation in Juvenile ALS Cannot be Ignored

Li Chen

doi:10.26717/bjstr.2021.34.005571

Abstract

Li Chen* and Jiajun Yang* Author Affiliations Department of Neurology, Shanghai Jiao Tong University Affiliated Sixth People’s Hospital, China *Both authors are equally contributed Received:March 04, 2021 | Published: March 15, 2021 Corresponding author:Li Chen and Jiajun Yang, Department of Neurology, Shanghai Jiao Tong University Affiliated Sixth People’s Hospital, Shanghai, 200233, China DOI: 10.26717/BJSTR.2021.34.005571

Highlights

Juvenile amyotrophic lateral sclerosis (JALS) is a degenerative neurological disease that occurs before the age of 25, involves upper and lower motor neurons and progresses progressively, with earlier onset, more rapid progression, and severe symptoms than classical ALS
Fused in sarcoma (FUS), which accounts for less than 1% of the causative mutation in classical ALS, is more than 60% of JALS, especially sporadic JALS [1,2]
It consists of seven domains, including a prion-like domain (PRLD) located at the N-terminal, three intrinsically disordered Arg-gly-rich domains (RGGs), one RNA recognition motif (RRM), and one RNA-binding zinc finger (ZNF) and a C-terminal nuclear localization signal (NLS) [1]

Summary

Introduction

The Pathological Role of Loss-Function of FUSNLS Mutation in Juvenile ALS Cannot be Ignored. The pathogenesis of JALS is still unknown and involves genetic, environmental, and biological factors. With the development of gene sequencing technology genetic factors are still dominate pathogenesis.

Results

Conclusion