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Transgene Detail
Symbol: Tg(NEFH)200Jpj
Name: transgene insertion 200, Jean-Pierre Julien
MGI ID: MGI:2182068
Synonyms: NF-H43
Transgene: Tg(NEFH)200Jpj  Location: unknown  
Strain of Origin:  Not Specified
Transgene Type:    Transgenic (Humanized sequence, Inserted expressed sequence)
Mutation:    Insertion
Tg(NEFH)200Jpj expresses 1 gene
Mutation detailsThe complete human NFH gene including 9.6 kb of 5' sequences and 13.4 kb of 3' sequences, coding for 43 Lys-Ser-Pro (KSP) phosphorylation sites, was used for the transgene. Expression of the human gene in transgenic animals was confirmed by Western blot analysis. (J:69179, J:69180)
View phenotypes for all genotypes (concatenated display).
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Carrying this Mutation:  Mouse Strains: 0 strains available      Cell Lines: 0 lines available
Founder 200 is representative of 2 founders carrying 14 and 20 copies of the transgene. These animals express approximately 1.5x - 4x the amount of endogenous mRNA and develop neurological anomalies after the first few weeks of life with progressive severity.

The phenotype is more severe in homozygous mice verses hemizygous mice, implying a correlation between phenotype and expression levels of NEFH.

Original:  J:69180 Cote F, et al., Progressive neuronopathy in transgenic mice expressing the human neurofilament heavy gene: a mouse model of amyotrophic lateral sclerosis. Cell. 1993 Apr 9;73(1):35-46
All:  11 reference(s)

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