mortality/aging
• 60% dead by 3 days after birth
|
adipose tissue
• complete absence of subcutaneous fat at the nape of the neck
|
• amount of subcutaneous fat is prominently reduced
• adipose deficiency and atrophy observed in multiple tissues during neonatal period
|
• adipocytes have fewer lipid cytoplasmic vacuoles than wild-type
• interscapular brown fat adipocytes are atrophic in neonatal (2-day old) pups
|
• interscapular brown fat adipocytes are atrophic in neonatal (2-day old) pups
|
behavior/neurological
growth/size/body
hematopoietic system
• effacement of normal thymic microarchitecture is observed in neonatal animals (2 days of age)
|
• poorly defined in 2-day old pups due to loss of normal thymic architecture
|
• poorly defined in 2-day old pups due to loss of normal thymic architecture
|
• spleen shows effacement of the normal architecture due to lymphoid depletion in white pulp
|
• severe lymphoid depletion
|
homeostasis/metabolism
hypoglycemia
(
J:81485
)
• serum glucose is about half of that in wild-type animals at 2 days postnatal
|
• hepatic glycogen stores are lost
• glycogen is absent from liver in 2-day old animals but is present prenatally
|
• glycogen is absent from skeletal muscle in 2-day old animals but is present prenatally
|
• increased expresssion of IL-1beta and IL-6 in skin and skeletal muscle
|
• increased expression in skin and skeletal muscle
• elevated serum levels
|
immune system
• effacement of normal thymic microarchitecture is observed in neonatal animals (2 days of age)
|
• poorly defined in 2-day old pups due to loss of normal thymic architecture
|
• poorly defined in 2-day old pups due to loss of normal thymic architecture
|
• spleen shows effacement of the normal architecture due to lymphoid depletion in white pulp
|
• severe lymphoid depletion
|
• increased expresssion of IL-1beta and IL-6 in skin and skeletal muscle
|
• increased expression in skin and skeletal muscle
• elevated serum levels
|
liver/biliary system
• hepatic glycogen stores are lost
• glycogen is absent from liver in 2-day old animals but is present prenatally
|
• atrophic, with microvesicular vacuolization, consistent with depletion of glycogen stores
|
muscle
• in 2-day old animals, increase in apoptotic cells is observed; in older pups, myofiber breakdown is observed
|
• glycogen is absent from skeletal muscle in 2-day old animals but is present prenatally
|
skeleton
• at E14, homozygotes display premature osteoblast differentiation and vascular invasion in skeletal elements ossifying through intramembranous (clavicles) or endochondral mechanism (ribs)
• at E14, cuboidal osteocalcin-expressing osteoblasts are organized in bone collar-like structures around the developing ribs in homozygous null but not in wild-type embryos
|
integument
• complete absence of subcutaneous fat at the nape of the neck
|
sparse hair
(
J:81485
)
• distorted shape
|
• dermal layer is atrophic due to reduction in adipose tissue leading to striking decrease in dermal thickness
|
• atrophy with hyperkeratosis in the epidermis
|
• atrophy with hyperkeratosis in the epidermis
|
cellular
• at E14, homozygotes display premature osteoblast differentiation and vascular invasion in skeletal elements ossifying through intramembranous (clavicles) or endochondral mechanism (ribs)
• at E14, cuboidal osteocalcin-expressing osteoblasts are organized in bone collar-like structures around the developing ribs in homozygous null but not in wild-type embryos
|
endocrine/exocrine glands
• effacement of normal thymic microarchitecture is observed in neonatal animals (2 days of age)
|
• poorly defined in 2-day old pups due to loss of normal thymic architecture
|
• poorly defined in 2-day old pups due to loss of normal thymic architecture
|