Analysis Tools
mortality/aging
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• a few hours after birth
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homeostasis/metabolism
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• in fibroblasts exposed to an LXR agonist
• unlike wild-type cells, fibroblasts accumulate lipid in the presence or absence of the lipid donor acetylated LDL
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dehydration
(
J:161652
)
|
• severe
|
|
• at E18.5
|
|
• glucosylceramide in the epidermis is increased greater than 2.9-fold compared to in wild-type mice
• unlike wild-type cells, fibroblasts accumulate lipid in the presence or absence of the lipid donor acetylated LDL
• however, mice exhibit normal total levels of phosphatidylinositols, phosphatidylethanolamines, phosphatidylcholines, acyl- and lyso-phosphatidylcholines and sphingomyelin
|
|
• in the epidermis
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growth/size/body
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• at E18.5
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behavior/neurological
cellular
|
• in fibroblasts exposed to an LXR agonist
• unlike wild-type cells, fibroblasts accumulate lipid in the presence or absence of the lipid donor acetylated LDL
|
integument
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• at E18.5
|
|
• from E16.5 onward, mice lack skin folds around the trunk and limbs unlike wild-type mice
|
|
• mice lack of dense palisade basal cell nuclear architecture compared with wild-type mice
|
|
• corneocyte envelopes are smaller, sparser, and more fragile than in wild-type mice
• mice exhibit reduced lipid deposition between the intercellular spaces of the corneocyte envelope compared with wild-type mice
|
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• from E16, mice exhibit hyperkeratosis with a 20 to 30 cell layer thick stratum corneum with unlike in wild-type mice
|
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• scarce or malformed
|
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• as a whole at E17.5 and P1
|
tight skin
(
J:161652
)
|
• the epidermis undergoes premature differentiation compared to in wild-type mice
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| autosomal recessive congenital ichthyosis 4B | DOID:0060713 |
OMIM:242500 |
J:161652 | |
