Tg(Prnp-TARDBP*Q331K)103Dwc
Transgene Detail

Summary

• Symbol: Tg(Prnp-TARDBP*Q331K)103Dwc
• Name: transgene insertion 103, Don W Cleveland
• MGI ID: MGI:5463119
• Synonyms: Prp-TDP-43^Q331K
• Transgene: Tg(Prnp-TARDBP*Q331K)103Dwc Location: unknown
• Alliance: Tg(Prnp-TARDBP*Q331K)103Dwc page

Transgene origin

• Strain of Origin: C57BL/6 x C3H

Transgene description

• Transgene Type: Transgenic (Humanized sequence, Inserted expressed sequence)
• Mutation: Insertion
• Tg(Prnp-TARDBP*Q331K)103Dwc expresses 1 gene
  Transgene expresses:
  

  Organism

  Expressed Gene

  Homolog in Mouse

  Note

  human

  TARDBP (23435)

  Tardbp

  (MGI:2387629)

• Mutation details: A full-length human TAR DNA binding protein (TARDBP or TDP-43) cDNA sequence was modified to have both an N-terminal myc tag and the glutamine to lysine substitution at amino acid 331 associated with familial ALS (huTDP-43*Q331K). This huTDP-43*Q331K cDNA sequence was inserted between exon 2 and exon 3 of mouse prion protein (Prnp) gene. The resulting Prnp-TDP-43*Q331K transgene was flanked with loxP sites. Founder line 103 was identified with approxiamately 2.5-fold greater total TDP-43 (1.5-fold greater huTDP-43*Q331K) expression levels in spinal cord as compared to endogenous TDP-43 in non-transgenic mice. (J:191785)

Disease models

Expression

In Structures Affected by this Mutation:

5 anatomical structure(s)

Find Mice (IMSR)

• Mouse strains and cell lines available from the International Mouse Strain Resource (IMSR)
• Carrying this Mutation: Mouse Strains: 1 strain available Cell Lines: 0 lines available

References

• Original: J:191785 Arnold ES, et al., ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43. Proc Natl Acad Sci U S A. 2013 Feb 19;110(8):E736-45
• All: 12 reference(s)