Effects of dietary cholesterol restriction in a feline model of Niemann-Pick type C disease.
Effects of dietary cholesterol restriction in a feline model of Niemann-Pick type C disease.
复制标题
饮食胆固醇限制对尼曼-匹克 C 型疾病猫模型的影响。
DOI：
10.1023/a:1010588112003
复制
发表时间：
2001
影响因子：
4.2
通讯作者：
Thrall,MA
中科院分区：
文献类型：
--
作者：
Somers,KL;Brown,DE;Fulton,R;Schultheiss,PC;Hamar,D;Smith,MO;Allison,R;Connally,HE;Just,C;Mitchell,TW;Wenger,DA;Thrall,MA
A feline model of Niemann–Pick disease type C (NPC) was employed to evaluate the effect of dietary cholesterol restriction on progression of disease. Two NPC‐affected treated cats were fed a cholesterol‐restricted diet beginning at 8 weeks of age; the cats remained on the diet for 150 and 270 days respectively. The study goal was to lower the amount of low density lipoprotein (LDL) available to cells, hypothetically reducing subsequent lysosomal accumulation of unesterified cholesterol and other lipids. Neurological progression of disease was not altered and dietary cholesterol restriction did not significantly decrease storage in NPC‐affected treated cats. One NPC‐affected treated cat had decreased serum alkaline phosphatase activity (ALP) and decreased serum cholesterol concentration. Liver lipid concentrations of unesterified cholesterol, cholesterol ester and phospholipids in NPC‐affected treated cats were similar to those seen in NPC‐affected untreated cats. Ganglioside concentrations in the NPC‐affected treated cats and NPC‐affected untreated cats were similar. Histological findings in liver sections from NPC‐affected treated cats showed a diffuse uniform microvacuolar pattern within hepatocytes and Kupffer cells, in contrast to a heterogeneous macro/microvacuolar pattern and prominent nodular fibrosis in NPC‐affected untreated cats. Similar differences in vacuolar patterns were seen in splenic macrophages. Although some hepatic parameters were modified, dietary cholesterol restriction did not appear to alter disease progression in NPC‐affected kittens.
