Mouse Genome Informatics
cx1
    Hsf1tm1Ijb/Hsf1tm1Ijb
Tg(TTR-V30M)15Imeg/0

involves: 129S6/SvEvTac * BALB/c * C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
nervous system
N
• as in human patients with familial amyloidotic polyneuropathy, no transthyretin deposition is observed in the brain and spinal cord (J:156658)
• after 24 months, mice exhibit a decrease in the number of unmyelinated fibers and myelinated fibers in sciatic nerves with transthyretin depositions unlike in wild-type mice
• at 3 months, some mice exhibit transthyretin depositions in the dorsal root ganglion unlike wild-type or Tg(TTR-V30M)15Imeg mice
• penetrance of thranthyretin increases with age
• mice exhibit transthyretin depositions in the sciatic nerve unlike wild-type or Tg(TTR-V30M)15Imeg mice
• at 24 months, mice exhibit collagen pockets indicating neurodegeneration unlike in Tg(TTR-V30M)15Imeg mice

digestive/alimentary system
• from 1 to 24 months of age, mice exhibit transthyretin depositions in the gastrointestinal tract unlike in wild-type mice
• mice exhibit a 2- to 3-fold higher penetrance of transthyretin deposition compared with Tg(TTR-V30M)15Imeg mice
• at 6 months, mice exhibit amyloid depositions in the stomach unlike wild-type mice
• a higher number of older mice exhibit amyloid depositions in the stomach

immune system
• in sciatic nerves and neurons of ganglion that exhibit transthyretin deposition
• in sciatic nerves and neurons of ganglion that exhibit transthyretin deposition

integument
• at 3 months, mice exhibit transthyretin depositions in the skin unlike wild-type or Tg(TTR-V30M)15Imeg mice

Mouse Models of Human Disease
OMIM IDRef(s)
Amyloidosis, Hereditary, Transthyretin-Related 105210 J:156658


Mouse Genome Informatics
tg2
    Tg(TTR-V30M)15Imeg/0
Not Specified
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
homeostasis/metabolism
• amyloid deposition starts in the gastrointestinal tract, cardiovascular system, and kidneys 6 months after birth and extends to various other organs and tissues with advancing age
• however, amyloid does not accumulate in the choroid plexus and in the peripheral and autonomic nervous systems

Mouse Models of Human Disease
OMIM IDRef(s)
Amyloidosis, Hereditary, Transthyretin-Related 105210 J:80880