| First Author | Muralidhar A | Year | 2011 |
| Journal | Mol Genet Metab | Volume | 103 |
| Issue | 2 | Pages | 142-7 |
| PubMed ID | 21459030 | Mgi Jnum | J:172067 |
| Mgi Id | MGI:5003388 | Doi | 10.1016/j.ymgme.2011.03.001 |
| Citation | Muralidhar A, et al. (2011) Pulmonary function and pathology in hydroxypropyl-beta-cyclodextin-treated and untreated Npc1(-/-) mice. Mol Genet Metab 103(2):142-7 |
| abstractText | 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. |
