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Histochemical and cellular changes accompanying the appearance of lung fibrosis in an experimental mouse model for Hermansky Pudlak syndrome
Hermansky Pudlak syndrome (HPS) is a heterogeneous recessive genetic disease with a tendency to develop lung fibrosis with aging. A mouse strain with two mutant HPS genes affecting separate vesicle trafficking pathways, C57BL/6-Hps1 (ep)-Ap3b1 (pe), exhibits severe lung abnormalities at young ages,...
| Autores principales: | , |
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| Formato: | Texto |
| Lenguaje: | English |
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Springer-Verlag
2010
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| Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2909458/ https://www.ncbi.nlm.nih.gov/pubmed/20603711 http://dx.doi.org/10.1007/s00418-010-0724-8 |