B6-3*hSMN2
Product Type
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Genotype
Sex
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Basic Information
Model Name
B6-3*hSMN2
Product ID
C001681
Gene Name
Background
C57BL/6NCya
Gene Alias
SMNC; BCD541; GEMIN1; TDRD16B; C-BCD541
Classification
Full-Gene Humanized Models,Neurological Disease Models,Rare Disease Models
NCBI ID
Phenotype
Chromosome
5
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Note: When using this mouse strain in a publication, please cite “B6-3*hSMN2 mice (Catalog C001681) were purchased from Cyagen.”
Strain Description
B6-3*hSMN2 mice are a humanized disease model carrying three copies of the human SMN2 gene, which can be used to mimic SMA patients with three SMN2 gene copies. Since the SMN2 gene primarily produces SMNΔ7 protein lacking exon 7, rather than full-length SMN protein, the humanized SMN2 gene cannot fully compensate for the abnormalities caused by Smn1 deficiency, resulting in the manifestation of SMA-like phenotypes in this model.
Application Area
Pathogenic mechanism of spinal muscular atrophy (SMA) and the preclinical evaluation of therapeutic drugs
Validation Data