Phenotypes associated with this allele
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Adam17tm1Imx mutation
(0 available);
any
Adam17 mutation
(62 available)
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immune system
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• in a model of sterile peritonitis irradiated, wild-type mice reconstituted with fetal liver cells exhibit accelerated neutrophil recruitment compared to in wild-type mice reconstituted with wild-type cells
• in vivo, irradiated wild-type mice reconstituted with fetal liver cells exhibit decreased leukocyte rolling velocity and increased adhesion compared to in wild-type mice reconstituted with wild-type cells
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homeostasis/metabolism
cellular
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• in a model of sterile peritonitis irradiated, wild-type mice reconstituted with fetal liver cells exhibit accelerated neutrophil recruitment compared to in wild-type mice reconstituted with wild-type cells
• in vivo, irradiated wild-type mice reconstituted with fetal liver cells exhibit decreased leukocyte rolling velocity and increased adhesion compared to in wild-type mice reconstituted with wild-type cells
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hematopoietic system
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• in a model of sterile peritonitis irradiated, wild-type mice reconstituted with fetal liver cells exhibit accelerated neutrophil recruitment compared to in wild-type mice reconstituted with wild-type cells
• in vivo, irradiated wild-type mice reconstituted with fetal liver cells exhibit decreased leukocyte rolling velocity and increased adhesion compared to in wild-type mice reconstituted with wild-type cells
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Adam17tm1Imx mutation
(0 available);
any
Adam17 mutation
(62 available)
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mortality/aging
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• a few animals survived for 3 weeks after birth
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• most animals died between E17.5 and P1
• most animals found alive at birth died within several hours
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pigmentation
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• irregular and dense pigmentation patterns within pelage hairs
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embryo
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• delayed epithelial maturation of the intestine, lung, nonglandular stomach, thyroid, parathyroid, and salivary gland
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• mild attenuation of the spongiotrophoblast layer associated with a disorganized and discontinuous plate of giant trophoblast cells
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• attenuation of the spongiotrophoblast layer
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growth/size/body
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• a >2-fold increase in fetal heart size, including both atria and ventricles, was observed after E18.5
• cardiac enlargement reached a maximum on P1, when most mutant mice died
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• at E18.5, homozygotes showed a 20%-30% increase in heart weight-to-body weight ratio relative to wild-type or heterozygous mutant mice
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• the few animals that survived weighed 20%-40% less than wild-type
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immune system
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• surviving animals had corneal inflammation
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vision/eye
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• surviving animals had corneal inflammation
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• surviving animals show eye degeneration
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• lacked a conjunctival sac
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• at E17.5, all fetuses had open eyelids due to failure of eyelid fusion
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• all newborns were readily identified by their open eyelids
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cardiovascular system
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• at P1, mutant cardiomyocytes were enlarged and were more loosely packed, with larger extracellular spaces
• however, no excessive collagen deposits or chamber septation abnormalities were observed
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• at E18.5, the enlarged ventricles and thickened myocardium displayed deep trabecular recesses
• enlarged fetal hearts with increased myocardial trabeculation and reduced cell compaction resemble pathological changes of noncompaction of ventricular myocardium
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• exhibit a ventricular septal defect
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• a >2-fold increase in fetal heart size, including both atria and ventricles, was observed after E18.5
• cardiac enlargement reached a maximum on P1, when most mutant mice died
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• at E18.5, homozygotes showed a 20%-30% increase in heart weight-to-body weight ratio relative to wild-type or heterozygous mutant mice
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• at E18.5, the ventricular wall, esp. the trabecular layer, was thicker than wild-type
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• at P1, the cell proliferation index in mutant hearts was 14 3% versus 8 1% in wild-type hearts; no differences were observed at E16.5
• no significant differences in cell apoptosis were noted in mutant hearts at P1
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muscle
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• at P1, mutant cardiomyocytes were enlarged and were more loosely packed, with larger extracellular spaces
• however, no excessive collagen deposits or chamber septation abnormalities were observed
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• at E18.5, the enlarged ventricles and thickened myocardium displayed deep trabecular recesses
• enlarged fetal hearts with increased myocardial trabeculation and reduced cell compaction resemble pathological changes of noncompaction of ventricular myocardium
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• at P1, the cell proliferation index in mutant hearts was 14 3% versus 8 1% in wild-type hearts; no differences were observed at E16.5
• no significant differences in cell apoptosis were noted in mutant hearts at P1
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digestive/alimentary system
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• proximal small intestine shows variably blunted villi and a hypercellular, pseudostratified mucosal epithelium displaying less cell polarity, at E17.5
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respiratory system
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• E17.5 embroys show impaired differentiation of the bronchiolar epithelium
• bronchioles are lined by epithelium that appears disorganized with variable hypercellularity, segmental stratification, and increased nuclear to cytoplasmic ratio
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integument
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• irregular and dense pigmentation patterns within pelage hairs
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• surviving animals had irregular hair follicle structure and distribution, hair follicles extended into adipose layer
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• surviving animals had irrigular hair follicle orientation
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• surviving animals had curly vibrissae
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cellular
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• at P1, the cell proliferation index in mutant hearts was 14 3% versus 8 1% in wild-type hearts; no differences were observed at E16.5
• no significant differences in cell apoptosis were noted in mutant hearts at P1
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Adam17tm1Imx mutation
(0 available);
any
Adam17 mutation
(62 available)
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Adam17tm1Imx/Adam17tm1Imx embryos develop internal hemorrhage
cardiovascular system
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• at E17.5, hemorrhaging is accompanied by a loss of the vascular organization
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• at E14.5, the large cranial vessels projecting posteriorly over the midbrain region show disruption of the elaborate branching pattern observed in controls, with shorter and less elaborate branching and abnormal fusion of vessels at various branch points not observed in controls
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• newborn homozygotes displayed enlarged atrioventricular valves
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• newborn homozygotes displayed enlarged semilunar valves
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• at P1, the mean area of mutant aortic valves was 57.5 +/- 29.4 mm2 relative to 20.0 +/- 7.4 mm2 observed in wild-type aortic valves
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• 50% and 67% of E14.5 and E17.5 embryos, respectively, exhibit internal hemorrhaging, however no hemorrhagic lesions on the yolk sack vasculature are seen
• hemorrhaging between E11.5 and E14.5 localizes primarily on the side of the head and extent and localization of bleeding progresses with age
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integument
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• at E17.5, mutants show signs of edema on the neck and the upper back area
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growth/size/body
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• at E17.5, mutants show signs of edema on the neck and the upper back area
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• fetuses at E14.5 and E17.5 tend to be shorter in length compared to controls, however the differences are not significant
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• fetuses at E14.5 and E17.5 tend to be underweight compared to controls, however the differences are not significant
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homeostasis/metabolism
mortality/aging
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• 50% die at E17.5, however no mortality is seen at E11.5 or E14.5
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nervous system
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• at E14.5, the large cranial vessels projecting posteriorly over the midbrain region show disruption of the elaborate branching pattern observed in controls, with shorter and less elaborate branching and abnormal fusion of vessels at various branch points not observed in controls
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Adam17tm1Imx mutation
(0 available);
any
Adam17 mutation
(62 available)
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mortality/aging
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• mice die shortly after birth due to respiratory failure
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vision/eye
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• fetuses (E16.5 or older) display open eyelids
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• at P0, newborn mice are reliably identified by an open eyelid phenotype
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homeostasis/metabolism
respiratory system
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• neonatal lungs have a semitranslucent gross appearance
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• the peripheral lung vascular network is underdeveloped, as determined by reduced PECAM-1 staining
• only sporadic PECAM-1-positive endothelial cells are found in thickened lung mesenchyme
• at E16.5, mutant lungs have ~60% fewer capillary branch points than control lungs
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• at P0, large and dilated airways are observed in the lung periphery, with fewer septations and thickened interstitial tissue, indicating delayed lung development
• PCNA proliferative indices are reduced by 64% and 40% at E16.5 and E18, respectively, in the bronchial epithelium
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• at E16.5, sparse air spaces with abundant mesenchyme reminiscent of the early pseudoglandular stage are seen, unlike in wild-type lungs where a normal branching pattern with regularly shaped terminal tubules is observed
• when placed in serum-free organ culture, E12 lung primordia from mutant mice branch poorly with only 51.5% the number of peripheral terminal buds seen in wild-type controls
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• at E18, fewer saccular structures, thickened interstitial mesenchyme and no septal formation are observed, instead of the characteristic thin-walled terminal buds with extending septa seen in wild-type lungs
• at E16.5 and E18, mutant lungs display 56% and 38% less distal airway spaces relative to wild-type lungs
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• a thickened lung mesenchymal tissue is observed at E18 and P0
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• fewer PCNA staining-positive epithelial cells are detected at E16.5 and E18
• PCNA proliferative indices are reduced by 64% and 40% at E16.5 and E18, respectively
• at E16.5, a reduced SP-C immunostaining intensity is detected in the fewer distal epithelial branches indicating a delay in (prealveolar type II) epithelial cell differentiation
• only weak SP-C staining is seen in some peripheral epithelial cells of dilated airways in cultured E12 mutant lungs
• both lung hypoplasia and delayed epithelial cell differentiation can be rescued by exogenous addition of soluble stimulatory factors including TNF or EGF in embryonic lung culture
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• neonatal lungs are hypoplastic
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• at P0, fewer, but dilated, terminal airways are observed in the lung periphery
• fewer airways of irregular shape are also seen in the proximal area with apparently thickened mesenchymal tissue
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• at P0, large terminal airways are dilated
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• neonatal lungs are pale in color
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• newborn (P0) mice show obvious breathing difficulties
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cardiovascular system
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• the peripheral lung vascular network is underdeveloped, as determined by reduced PECAM-1 staining
• only sporadic PECAM-1-positive endothelial cells are found in thickened lung mesenchyme
• at E16.5, mutant lungs have ~60% fewer capillary branch points than control lungs
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Adam17tm1Imx mutation
(0 available);
any
Adam17 mutation
(62 available)
Adam17woe mutation
(0 available);
any
Adam17 mutation
(62 available)
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vision/eye
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• authors state that mice exhibit similar phenotypes as observed in Adam17woe homozygotes
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integument
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• authors state that mice exhibit similar phenotypes as observed in Adam17woe homozygotes
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Adam17tm1Imx mutation
(0 available);
any
Adam17 mutation
(62 available)
Adam19Gt(Betageo)1Bbl mutation
(1 available);
any
Adam19 mutation
(77 available)
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mortality/aging
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• die around E13.5 to E14.5
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cardiovascular system
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• 6 of 7 embryos at E13.5-14.5 show an underdeveloped myocardial wall, especially of the left ventricle
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• 6 of 7 embryos at E13.5-14.5 show severe lack of trabeculation
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muscle
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• 6 of 7 embryos at E13.5-14.5 show an underdeveloped myocardial wall, especially of the left ventricle
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• 6 of 7 embryos at E13.5-14.5 show severe lack of trabeculation
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Adam12tm1Asf mutation
(0 available);
any
Adam12 mutation
(41 available)
Adam15tm1Bbl mutation
(0 available);
any
Adam15 mutation
(36 available)
Adam17tm1Imx mutation
(0 available);
any
Adam17 mutation
(62 available)
Adam9tm1Bbl mutation
(1 available);
any
Adam9 mutation
(44 available)
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mortality/aging
cardiovascular system
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• thickened and misshapen valves
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• thickened and misshapen valves
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• thickened and misshapen valves
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• thickened and misshapen valves
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vision/eye
Analysis Tools