B6-hFUS*R521C
Product Type
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Genotype
Sex
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Basic Information
Model Name
B6-hFUS*R521C
Product ID
C001647
Gene Name
Background
C57BL/6JCya
Gene Alias
TLS; ALS6; ETM4; FUS1; POMP75; altFUS; HNRNPP2
Classification
Full-Gene Humanized Models,Neurological Disease Models,Rare Disease Models
NCBI ID
Phenotype
Chromosome
16
Document
Note: When using this mouse strain in a publication, please cite “B6-hFUS*R521C mice (Catalog C001647) were purchased from Cyagen.”
Strain Description
This model is a humanized model. Gene editing technology is used to replace the endogenous mouse Fus gene with a human FUS gene fragment carrying the R521C mutation. B6-hFUS*R521C mice can be used for the research of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration/dementia (FTLD).
Application Area
Research on amyotrophic lateral sclerosis (ALS)
Research on frontotemporal lobar degeneration/dementia (FTLD-FUS)
Validation Data