behavior/neurological
hyperactivity
(
J:80556
)
growth/size/body
• 2-fold decrease in body weight at time of weaning but fully recover weight as adults
|
• growth retardation before weaning which becomes discernible at 5 days after birth
|
hematopoietic system
• impairment of pre-B cell maturation in the bone marrow
|
• the type 1 transitional B cell fraction is modestly decreased to 31% of the total B cell population in the spleen, compared with 38% in wild-type
|
• near complete absence of B cells in small intestines
• 40% reduction in lymph node B cells compared to wild-type
• 65% reduction in splenic B cells compared to wild-type
|
• severe reduction in the pool of pre-B cell numbers
|
small spleen
(
J:80204
)
• spleen weight is 40% of wild-type weight
|
• B cell follicles are virtually absent
|
• serum IgM levels are significantly reduced in suckling mice and after weaning
|
immune system
• impairment of pre-B cell maturation in the bone marrow
|
• the type 1 transitional B cell fraction is modestly decreased to 31% of the total B cell population in the spleen, compared with 38% in wild-type
|
• near complete absence of B cells in small intestines
• 40% reduction in lymph node B cells compared to wild-type
• 65% reduction in splenic B cells compared to wild-type
|
• severe reduction in the pool of pre-B cell numbers
|
small spleen
(
J:80204
)
• spleen weight is 40% of wild-type weight
|
• B cell follicles are virtually absent
|
• serum IgM levels are significantly reduced in suckling mice and after weaning
|
• macroscopically absent although marker analysis indicates that normal numbers of Peyer's patch anlagen develop after the first postnatal week
(J:80204)
• arginine injections restore development of Peyer's patches to normal
(J:80204)
• Peyer's patch formation is defective, with mutants exhibiting fewer and smaller Peyer's patches protruding from the serosa of the small intestine at 18 days of age
(J:80556)
|
muscle
• diameter of gastrocnemius myofibers is about 1/3 that of wild-type, with many nuclei located in the periphery of the fibers
|
homeostasis/metabolism
• in suckling mice, plasma and jejunal arginine concentrations are reduced (recovers after weaning) while enterocyte concentration is increased compared to that in wild-type
|
• in suckling mice, plasma and jejunal arginine concentrations are reduced (recover after weaning)
|
• suckling mice show a decrease in circulating plasma arginine concentration due to intestinal arginase activity
|
• 90% increase in plasma glycine concentration in the first postnatal week and to 25% and 15% in the second and third weeks
|
• 30% increase in the arginase product ornithine in the jejunum
|
• intestinal arginase activity is increased compared to wild-type
|
digestive/alimentary system
• expression analysis indicates that maturation of the enterocytes is slightly slower than in wild-type mice
|
integument
• appearance of a normal fur coat is delayed until after weaning
|
sparse hair
(
J:80556
)
• keratin plugs clogged the follicles
|
• severe distortion of hair follicles
• many hair bulbs are too high in the subcutaneous tissue, contain keratohyalin grains and do not develop properly
|
• absence of trichohyalin from the epidermis
|
• keratin plugs clog hair follicles
|