Publications

Latest publication 12/17/2021

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
Amyotrophic Lateral Sclerosis (ALS)
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A unique, automatic apparatus for the detailed and objective modeling of walking and coordination disorders in mice and/or rats, with respect to motor and psychomotor skills and cognition. An innovative and operator-independent solution for studies on myopathy, ataxia, alcoholism, Parkinson's and Huntington's disease, and much more.

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An easy way to objectively quantify the muscular strength of mice and rats, and to assess the effect of drugs, toxins, muscular (i.e. myopathy) and neurodegenerative diseases on muscular degeneration. It is widely used in conjunction with the ROTAROD motor coordination test: a normally coordinated rodent will show a decreased latency to fall off the rotating rod if its muscular strength is low. The Grip Strength Test is a must for your research on activity, motor control & coordination, and is particularly well suited for studies on Parkinson's & Huntington's disease.
New features GS4 - 2023: Color display with permanent backlight screen for easier reading, reset by footswitch, Improved battery time, Larger data memory of 500 values, Animal counter, USB port (charging/data transfer)

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