Analysis Tools
mortality/aging
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• mean lifespan is about 24 days although a few survive to 30-39 days of age
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growth/size/body
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• severely anemic mice may exhibit cardiomegaly
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scaly ears
(
J:11262
)
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• crusting of the ears by 14-15 days of age
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small ears
(
J:11262
)
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• ears are small and sometimes folded
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thick ears
(
J:11262
)
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• swollen abdomen
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• spleen weights are 2-4 times those of controls
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vision/eye
blepharitis
(
J:11262
)
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• scaliness on eyelids that seals the palpebral fissure
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hearing/vestibular/ear
scaly ears
(
J:11262
)
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• crusting of the ears by 14-15 days of age
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small ears
(
J:11262
)
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• ears are small and sometimes folded
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thick ears
(
J:11262
)
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reproductive system
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• swelling and reddening of the genital papilla at 12-14 days of age
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small testis
(
J:11262
)
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cryptorchism
(
J:11262
)
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• testicles are retained in the abdominal cavity
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immune system
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• the thymic cortex is rapidly depleted of lymphocytes over time
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small thymus
(
J:11262
)
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• a bilobed thymus is present but extremely small and is densely populated with lymphocytes
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• exhibit lymphoproliferative lesions
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• mild to moderate sinus histiocytosis in the lymph nodes
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• lesions in the spleen
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• spleen weights are 2-4 times those of controls
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• massively expanded by hematopoietic cells
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• white pulp may be enlarged or shrunken and is composed of blastlike mononuclear cells with vesicular nuclei, prominent reticulum cells, lymphoblasts, and variable number of plasma cells
• occasional erythrophagocytic macrophages and plasma cells with Russel's bodies can be seen at the margins of the white pulp
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• follicles are lacking and lymphocytes are absent in the spleen
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• distinct marginal zones are lacking
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• IgA synthesis is precocious, detectable at P21 unlike in controls
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• apparent as early as 10 days of age and ranges from 2-10 times the concentration in controls
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• lesions in the lymph nodes that contain a randomly distributed mixture of lymphoblasts, blastlike mononuclear cells with vesicular nuclei, hypertrophic reticulum cells, macrophages, and granulocytes instead of small lymphocytes
• complete loss of normal architecture
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• lack of discernible follicles
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• no distinct paracortex
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• thickening of the medullary cords
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• subcutaneous lymph nodes such as inguinal, axillary, and cervical nodes and consistently enlarged while visceral lymph nodes are slightly or moderately enlarged
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• perivascular infiltrates of mixed mononuclear cells and granulocytes are seen in heart, pancreas, lung, salivary gland, kidney, and mesenteries
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blepharitis
(
J:11262
)
|
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• scaliness on eyelids that seals the palpebral fissure
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• leukocytic infiltrations are present in the portal areas of the liver
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dermatitis
(
J:11262
)
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• a diffuse lymphohistiocytic infiltration of the entire dermis that is most severe in the prepuce, ears, eyelids and facial skin
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hematopoietic system
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• the thymic cortex is rapidly depleted of lymphocytes over time
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small thymus
(
J:11262
)
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• a bilobed thymus is present but extremely small and is densely populated with lymphocytes
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• abundant hematopoiesis in the hepatic sinusoids, spleen and bone marrow
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• mean hematocrit values are about one half those of controls
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• mean hemoglobin volumes are about one half those of controls
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anisocytosis
(
J:11262
)
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• exhibit lymphoproliferative lesions
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• mild to moderate sinus histiocytosis in the lymph nodes
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• lesions in the spleen
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• spleen weights are 2-4 times those of controls
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• massively expanded by hematopoietic cells
|
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• white pulp may be enlarged or shrunken and is composed of blastlike mononuclear cells with vesicular nuclei, prominent reticulum cells, lymphoblasts, and variable number of plasma cells
• occasional erythrophagocytic macrophages and plasma cells with Russel's bodies can be seen at the margins of the white pulp
|
|
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• follicles are lacking and lymphocytes are absent in the spleen
|
|
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• distinct marginal zones are lacking
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• IgA synthesis is precocious, detectable at P21 unlike in controls
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• apparent as early as 10 days of age and ranges from 2-10 times the concentration in controls
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liver/biliary system
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• leukocytic infiltrations are present in the portal areas of the liver
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• lesions in the liver
• occasionally livers have marked erythrophagocytosis or hemosiderin deposits in Kupffer cells
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• many mice have a thin, sharply demarcated rim of necrosis along the margins of the liver
• areas of acute coagulative necrosis without inflammation are present at the tips of liver lobes
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• centrolobular hepatic cords are atrophied
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homeostasis/metabolism
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• occasionally see acute right atrial thrombosis
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• severely anemic mice may exhibit pleural effusion
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hemosiderosis
(
J:11262
)
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• occasionally livers have marked hemosiderin deposits in Kupffer cells
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renal/urinary system
pale kidney
(
J:11262
)
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behavior/neurological
cardiovascular system
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• severely anemic mice may exhibit cardiomegaly
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endocrine/exocrine glands
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• the thymic cortex is rapidly depleted of lymphocytes over time
|
small thymus
(
J:11262
)
|
|
• a bilobed thymus is present but extremely small and is densely populated with lymphocytes
|
small testis
(
J:11262
)
|
|
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cryptorchism
(
J:11262
)
|
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• testicles are retained in the abdominal cavity
|
craniofacial
scaly ears
(
J:11262
)
|
|
• crusting of the ears by 14-15 days of age
|
small ears
(
J:11262
)
|
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• ears are small and sometimes folded
|
thick ears
(
J:11262
)
|
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integument
dermatitis
(
J:11262
)
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• a diffuse lymphohistiocytic infiltration of the entire dermis that is most severe in the prepuce, ears, eyelids and facial skin
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• occasionally see intraepidermal pustules
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• moderate to severe orthokeratotic hyperkeratosis
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parakeratosis
(
J:11262
)
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• multifocal parakeratosis
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scaly skin
(
J:11262
)
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• by 14-15 days of age, ears, feet, tail and eyelids are scaly
• scales on base of tail may form thick circumferential rings
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skin lesions
(
J:11262
)
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• apparent at 7 days of age
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• scales on base of tail may form thick circumferential rings
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respiratory system
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• severely anemic mice may exhibit pleural effusion
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cellular
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• many mice have a thin, sharply demarcated rim of necrosis along the margins of the liver
• areas of acute coagulative necrosis without inflammation are present at the tips of liver lobes
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| immune dysregulation-polyendocrinopathy-enteropathy-X-linked syndrome | DOID:0090110 |
OMIM:304790 |
J:66734 | |
| NOT | X-linked ichthyosis | DOID:1700 |
OMIM:308100 |
J:11262 |
