mortality/aging
• 20-30% survival to 7-10 days
• rare individuals survive to 15-18 days
|
behavior/neurological
limb grasping
(
J:83644
)
• clasp their hind limbs when lifted by the tail
|
• fall over readily
|
abnormal gait
(
J:83644
)
• abnormal gait seen in mice over 7-9 days of age
|
growth/size/body
microcephaly
(
J:83644
)
• severely growth retarded
• 5-10 day old mice only 20-35% the size of controls
|
homeostasis/metabolism
• cholesterol levels in liver and other tissue reduced about 40%
|
• cholesterol levels in liver reduced about 40%
|
liver/biliary system
• cholesterol levels in liver reduced about 40%
|
• increased fat content of the liver in the early postnatal period
|
cholestasis
(
J:83644
)
• abnormal bile acid formation may result in malabsorption syndrome
|
renal/urinary system
• renal dysfunction in terminal stages
|
nervous system
• brains are about 65% of control at 5-10 days
|
• delays in folial development detectable as early as age 3 days and persist to day 18
|
• delayed cellular accumulation and a slower cell loss
• thicker than normal at day 8 and gone at day 18 in the only mouse looked at
• migration of granule neurons both delayed and very slow
|
• irregularly shaped and not well aligned at day 8
• alignment improves at day 15 but still with an immature appearance
• slow maturation and less branching of dendrites
|
• layer thin at day 8 but normal at day 10
• impaired granule neuron survival
|
• cerebellar growth normal until age 5 days but slows down after day 7
|