Analysis Tools
mortality/aging
| N |
• despite ataxia and weight loss, mice exhibit normal lifespan
|
nervous system
|
• at P12, Purkinje cell dendritic arbors are shorter than in wild-type mice
• at P17, reduced arbor size is more pronounced than earlier with higher order branch complexity than in wild-type mice
• however, Purkinje cells are normal until 3 weeks of age
|
|
• progressive
|
|
• at 4 months of age
|
behavior/neurological
|
• beginning at P7
|
growth/size/body
|
• beginning at P7
|
weight loss
(
J:172719
)
|
• in adult mice
|
homeostasis/metabolism
|
• brain microsomes exhibit reduced C18 fatty acyl-CoA activity compared with wild-type microsomes
|
|
• the amount of total ceramide in the brain is reduced compared to in wild-type brains
• the amount of C14 ceramide in the brain is increased compared to in wild-type mice
• the amounts of C18 and C18:1 sphingomyelin in the brain are decreased compared to in wild-type mice
• the amounts of C14 and C16 dihydroceramide are increased while the amount of C18:1 dihydroceramide is decreased compared to in wild-type mice
• brains exhibit a 2- to 4-fold reduction in sphingosine and a more than 10-fold increase in dihydrosphingosine compared with wild-type mice
|
pigmentation
|
• in the brain
|
