Publications

Dernière publication 17/12/2021

Generation and analysis of innovative genomically humanised knock-in SOD1, TARDB

Amyotrophic lateral sclerosis/frontotemporal dementia (ALS/FTD) is a fatal neurodegenerative disorder, and continued innovation is needed for...

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    [title] => Generation and analysis of innovative genomically humanised knock-in SOD1, TARDB
    [paragraph] => Generation and analysis of innovative genomically humanised knock-in SOD1, TARDBP (TDP-43), and FUS mouse models
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Authors
A Devoy, G Price, F De Giorgio et al

 Lab
UK MRC Harwell Institute, Harwell Campus, Oxfordshire, UK
Journal
iScience
Abstract
Amyotrophic lateral sclerosis/frontotemporal dementia (ALS/FTD) is a fatal neurodegenerative disorder, and continued innovation is needed for improved understanding and for developing therapeutics. We have created next-genera tion genomically humanized knockin mouse models, by replacing the mouse genomic region of Sod1, Tardbp (TDP-43), and Fus, with their human orthologs, preserving human protein biochemistry and splicing with exons and introns intact. We establish a new standard of large knockin allele quality control, demon strating the utility of indirect capture for enrichment of a genomic region of interest followed by Oxford Nanopore sequencing. Extensive analysis shows that homozygous humanized animals only express human protein at endogenous levels. Characterization of humanized FUS animals showed that they are pheno typically normal throughout their lifespan. These humanized strains are vital for preclinical assessment of interventions and serve as templates for the addition of coding or non-coding human ALS/FTD mutations to dissect disease pathome chanisms, in a physiological context. 
BIOSEB Instruments Used
Automatic Foot misplacement apparatus (BIO-FMA),Grip strength test (BIO-GS3)

Keywords/Topics
Sclérose Latérale Amyotrophique (SLA), ou Maladie de Charcot
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Un test unique pour modéliser de façon précise les troubles de la marche disponible pour souris et/ou rats, pour des problèmes moteurs, psychomoteurs ou cognitifs. Une solution innovante et indépendante de l'opérateur pour vos études sur la myopathie, l'ataxie, l'alcoolisme, les maladies de Parkinson et d'Huntington, et bien plus.

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Nouveautés GS4 - 2023: Écran couleur rétroéclairé (meilleure lisibilité), pédale de remise à zéro, durée de batterie optimisée, taille de mémoire augmentée à 500 valeurs, compteur d'animaux, port USB (transfert de données/charge)

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