Tg(Notch3*R169C)88Bbb
Transgene Detail

Summary

• Symbol: Tg(Notch3*R169C)88Bbb
• Name: transgene insertion 88, Norbert Hubner
• MGI ID: MGI:4429538
• Synonyms: TgNotch3^R169C
• Transgene: Tg(Notch3*R169C)88Bbb Location: unknown
• Alliance: Tg(Notch3*R169C)88Bbb page

Tg(Notch3*R169C)88Bbb/0 mice exhibit granular osmiophilic material deposits in smooth muscle cells

Show the 7 phenotype image(s) involving this allele.

Transgene origin

• Strain of Origin: FVB/N

Transgene description

• Transgene Type: Transgenic (Inserted expressed sequence)
• Mutation: Insertion
• Tg(Notch3*R169C)88Bbb expresses 1 gene
  Transgene expresses:
  

  Organism

  Expressed Gene

  Homolog in Mouse

  Note

  rat

  Notch3 (56761)

  Notch3

  (MGI:99460)

• Mutation details: The transgene contains a modified rat BAC RPCI31.78K09 with the rat Notch3 gene carrying the CADASIL-associated mutation R169C. Two lines were created (88 and 92). Line 88 exhibit a 4-fold increase in expression and was selected as the representative line. (J:156682)

Disease models

Expression

In Structures Affected by this Mutation:

8 anatomical structure(s)

Find Mice (IMSR)

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

Notes

Phenotypic Similarity to Human Syndrome: Cerebral Ischemic Small Vessel Disease (J:156682)

References

• Original: J:156682 Joutel A, et al., Cerebrovascular dysfunction and microcirculation rarefaction precede white matter lesions in a mouse genetic model of cerebral ischemic small vessel disease. J Clin Invest. 2010 Feb 1;120(2):433-45
• All: 11 reference(s)