Pulmonary function and pathology in hydroxypropyl-beta-cyclodextin-treated and untreated Npc1-/- mice

被引：26

作者：

Muralidhar, Akshay ^{[1
]}

Borbon, Ivan A. ^{[1
]}

Esharif, Dyadin M. ^{[1
]}

Ke, Wangjing ^{[1
]}

Manacheril, Rinu ^{[1
]}

Daines, Michael ^{[1
,2
]}

Erickson, Robert P. ^{[1
,3
]}

机构：

[1] Univ Arizona, Hlth Sci Ctr, Dept Pediat, Tucson, AZ 85724 USA

[2] Univ Arizona, Arizona Resp Ctr, Tucson, AZ 85724 USA

[3] Univ Arizona, Dept Mol & Cellular Biol, Tucson, AZ 85724 USA

来源：

MOLECULAR GENETICS AND METABOLISM | 2011年 / 103卷 / 02期

关键词：

Niemann-Pick C1; Intracellular cholesterol transport; Foamy macrophages; Small animal ventilator; Hydroxypropyl-beta-cyclodextrins; NIEMANN-PICK-C; CHOLESTEROL-METABOLISM; SMOOTH-MUSCLE; EVERY ORGAN; MOUSE MODEL; DISEASE; TRANSPORT; LUNG; PROTEIN; OVERCOMES;

D O I：

10.1016/j.ymgme.2011.03.001

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Lung dysfunction is an important part of the pathology of the neurodegenerative disorder, Niemann-Pick C1 (NPC1). We have studied the pulmonary disease in the Npc1(NIH/NIH) mouse model. On histology, we find large numbers of alveolar foamy macrophages but no alveolar proteinosis. Lung weight as percent of body weight was markedly increased; using the flexiVent small animal ventilator (SCIREQ Inc.), we find inspiratory capacity, elastance and hysterisivity to be increased while resistance was not changed. Cholesterol measurements show a doubling of lung cholesterol levels. Collagen is also increased. Treatment of Npc1(-/-) mice with hydroxypropyl-beta-cyclodextrin (HPBCD), despite efficacious effects in brain and liver, results in little difference from age-matched controls (using a CNS-expressed transgene to extend the life expectancy of the Npc1(-/-) mice) for these variables. (C) 2011 Elsevier Inc. All rights reserved.

引用

页码：142 / 147

页数：6

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