期刊
MOLECULAR GENETICS AND METABOLISM
卷 103, 期 2, 页码 142-147出版社
ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.ymgme.2011.03.001
关键词
Niemann-Pick C1; Intracellular cholesterol transport; Foamy macrophages; Small animal ventilator; Hydroxypropyl-beta-cyclodextrins
资金
- NIH [5RO1 ED000343-5, 1R56 AI083403-01A1]
- Holsclaw Family Professorship of Human Genetics and Inherited Diseases
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.
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