RRC ID 80912
Author Eck RJ, Stair JG, Kraemer BC, Liachko NF.
Title Simple models to understand complex disease: 10 years of progress from Caenorhabditis elegans models of amyotrophic lateral sclerosis and frontotemporal lobar degeneration.
Journal Front Neurosci
Abstract The nematode Caenorhabditis elegans are a powerful model system to study human disease, with numerous experimental advantages including significant genetic and cellular homology to vertebrate animals, a short lifespan, and tractable behavioral, molecular biology and imaging assays. Beginning with the identification of SOD1 as a genetic cause of amyotrophic lateral sclerosis (ALS), C. elegans have contributed to a deeper understanding of the mechanistic underpinnings of this devastating neurodegenerative disease. More recently this work has expanded to encompass models of other types of ALS and the related disease frontotemporal lobar degeneration (FTLD-TDP), including those characterized by mutation or accumulation of the proteins TDP-43, C9orf72, FUS, HnRNPA2B1, ALS2, DCTN1, CHCHD10, ELP3, TUBA4A, CAV1, UBQLN2, ATXN3, TIA1, KIF5A, VAPB, GRN, and RAB38. In this review we summarize these models and the progress and insights from the last ten years of using C. elegans to study the neurodegenerative diseases ALS and FTLD-TDP.
Volume 17
Pages 1300705
Published 2023-1-1
DOI 10.3389/fnins.2023.1300705
PMID 38239833
PMC PMC10794587
C.elegans tm472 tm924 tm3262 tm1156 tm3690 tm2595 tm1380 tm1287 tm760 tm776 tm1146 tm1447 tm544 tm5202 tm1574 tm543 tm3673 tm4439 tm781 tm3335 tm3607 tm985 tm3120