respiratory system
• treatment with benzo(a)pyrene by intratracheal instillation leads to lung adenomas in 71% of homozygotes compared to 35% of controls
|
• in 10% of mice after treatment with benzo(a)pyrene by intratracheal instillation
|
growth/size/body
• the weight of males up to 2 years of age is reduced relative to that of controls
|
cellular
• fibroblasts from newborn mice are highly sensitive to UV radiation
(J:28709)
• higher inflammatory edema after UV-B exposure
(J:28709)
• skin ulcers form within 1-2 weeks of exposure
(J:28709)
• mice develop stronger and longer lasting ear swelling/inflammatory edema after ultraviolet B (UV-B) and topical psoralen plus ultraviolet A (PUVA) radiation indicating increased susceptibility to UV-B and PUVA radiation
(J:35054)
• abdominal skin shows intracellular edema and necrosis in the epidermis and subepidermal bullae at 24 hours after UV-B irradiation and mice show marked inflammatory infiltrates of lymphocytes, pronounced edema, vasodilation, and extravasation of erythrocytes in the dermis
(J:35054)
• mice develop enhanced sunburn cell formation after UV-B irradiation
(J:35054)
|
• defect in nucleotide excision repair
• failure to remove thymine dimers
|
neoplasm
• spontaneous tumors begin to appear at around 12 months
• spontaneous tumors are very abundant at 24 months
|
• in all mice by 34 weeks after UV-B exposure
|
• treatment with benzo(a)pyrene by intratracheal instillation leads to lung adenomas in 71% of homozygotes compared to 35% of controls
|
• in 10% of mice after treatment with benzo(a)pyrene by intratracheal instillation
|
• as a result of exposure to 9,10-dimethyl-1,2benz(a)anthracene (DMBA)
|
• increased sensitivity to the tumor forming effects of 9,10-dimethyl-1,2benz(a)anthracene (DMBA)
|
immune system
• UV-B-induced local immunosuppression and UV-B-induced systemic immunosuppression are enhanced when mice are sensitized with DNFB after UV-B irradiation
• however, mice develop contact sensitivity to DNFB similarly to controls
|
• daily UV-B exposure for 3 or 5 days results in a significantly reduced cell count
• slower recovery
|
• daily UV-B exposure for 3 or 5 days results in a significantly reduced cell count
• cell activity reduced to significantly below non irradiated levels by 1 day after exposure
• slower recovery
• reduced activity persists at least 5 days
• full recovery of activity by day 10
|
• mice show a greater loss of and morphologic damage of ADPase (+) Langerhans cells after UV-B irradiation
• recovery of Langerhans cells after UV-B irradiation is slower than for controls
|
reproductive system
• disorganization by 12 months
|
• some degeneration seen at 6 months
• by 12 months half of tubules have vacuoles in the spermatogenic epithelium
|
• at 24 months
|
• relative testis weight drops progressively over time
• down to 1/3 the weight of testes from control mice at 2 years
|
• in all seminiferous tubules by 24 months
|
• lack spermatozoa at 24 months
|
• males remain fertile until about 30 weeks
|
homeostasis/metabolism
• defect in nucleotide excision repair
• failure to remove thymine dimers
|
• in males at 24 months
|
• PGD2, PGE2 and PGF2alpha levels increase significantly after UV-B irradiation
|
• increased sensitivity to the tumor forming effects of 9,10-dimethyl-1,2benz(a)anthracene (DMBA)
|
hematopoietic system
• daily UV-B exposure for 3 or 5 days results in a significantly reduced cell count
• slower recovery
|
• daily UV-B exposure for 3 or 5 days results in a significantly reduced cell count
• cell activity reduced to significantly below non irradiated levels by 1 day after exposure
• slower recovery
• reduced activity persists at least 5 days
• full recovery of activity by day 10
|
endocrine/exocrine glands
• disorganization by 12 months
|
• some degeneration seen at 6 months
• by 12 months half of tubules have vacuoles in the spermatogenic epithelium
|
• at 24 months
|
• relative testis weight drops progressively over time
• down to 1/3 the weight of testes from control mice at 2 years
|
integument
• in all mice by 34 weeks after UV-B exposure
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
xeroderma pigmentosum group A | DOID:0110843 |
OMIM:278700 |
J:35054 |