Analysis Tools
nervous system
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• progressive reduction from age 1 month
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• vacuolization at age 1 month
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• vacuolization at age 1 month
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• at age P16, progressively thinning
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• at age P16
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• progressive reduction in thickness from age 1 month
• progressive increase in apoptotic (caspase 3 positive) cells
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• progressive loss of rod function
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vision/eye
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• normal retina vascularization
• normal retinal pigment epithelium morphology at age 1 month
• normal photoreceptor inner segment mitochondria number and morphology at age 1 month
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• numerous small white foci
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• progressive reduction from age 1 month
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• vacuolization at age 1 month
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• vacuolization at age 1 month
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• at age P16, progressively thinning
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• at age P16
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• progressive reduction in thickness from age 1 month
• progressive increase in apoptotic (caspase 3 positive) cells
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• progressive loss of rod function
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• progressive reduction from age 1 month
• multi-focal reduction in thickness to 2-4 (from 10-12 wildtype) rows at age 3 months
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• pyknotic cells
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• with scotopic and photopic ERG at age 3 months
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• with scotopic and photopic ERG at age 3 months
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mortality/aging
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• mice die at about 9 weeks instead of at 3 weeks as in Ptpn6me homozygotes
• death is due to a progressive and fatal autoimmune syndrome
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immune system
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• prothymocytes from mutant mice do not proliferate or differentiate due to a defect in activity of intrathymic accessory cells derived from bone marrow
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• lambda1 and lambda2, 3 B cells are reduced 14- and 4-fold, respectively, in the spleen and 5.9- and 2.2-fold, respectively, in the bone marrow compared to B cell numbers in wild-type mice
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• a result of a rapid expansion of the plasma cell population
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• atypical plasma or Mott cells with Russell bodies comprising crystallized immunoglobulin occur in lymphoid tissue
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• due to a factor in serum of mutant mice that increases cell maturation by three orders of magnitude
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• uncontrolled levels
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• mutant mice display a decreased response to T-cell mitogens
• interleukin 2 was unable to restore proliferation response in splenic cultures
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• resembles rheumatoid arthritis
• immunosuppressant drugs inhibit arthritic symptoms, but non-steroid anti-inflammatory drugs do not
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• reactions in the paws consist mainly of polymorphonuclear and mononuclear cell infiltration in the subcutaneous tissue extending to the periosteum and joint
• paws become deformed causing progressive problems walking
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• pneumonitis progression is slower than in Ptpn6me homozygotes but still causes death
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respiratory system
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• pneumonitis progression is slower than in Ptpn6me homozygotes but still causes death
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growth/size/body
hematopoietic system
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• prothymocytes from mutant mice do not proliferate or differentiate due to a defect in activity of intrathymic accessory cells derived from bone marrow
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• lambda1 and lambda2, 3 B cells are reduced 14- and 4-fold, respectively, in the spleen and 5.9- and 2.2-fold, respectively, in the bone marrow compared to B cell numbers in wild-type mice
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• a result of a rapid expansion of the plasma cell population
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• atypical plasma or Mott cells with Russell bodies comprising crystallized immunoglobulin occur in lymphoid tissue
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• due to a factor in serum of mutant mice that increases cell maturation by three orders of magnitude
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• uncontrolled levels
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• mutant mice display a decreased response to T-cell mitogens
• interleukin 2 was unable to restore proliferation response in splenic cultures
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pigmentation
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• at 3-4 days of age mice have focal depigmentation of the skin
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skeleton
integument
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• patchy absence of hair follows focal skin depigmentation giving the coat an uneven or motheaten appearance
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• necrotic lesions can develop on paws, tail and ears
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• at 3-4 days of age mice have focal depigmentation of the skin
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endocrine/exocrine glands
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• prothymocytes from mutant mice do not proliferate or differentiate due to a defect in activity of intrathymic accessory cells derived from bone marrow
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