Aberrant lung structure, composition, and function in a murine model of Hermansky-Pudlak syndrome.

  title={Aberrant lung structure, composition, and function in a murine model of Hermansky-Pudlak syndrome.},
  author={Timothy A. Lyerla and Michael E. Rusiniak and Michael Borchers and Gerald P. Jahreis and Jian Tan and Patricia J Ohtake and Edward K. Novak and Richard T. Swank},
  journal={American journal of physiology. Lung cellular and molecular physiology},
  volume={285 3},
Hermansky-Pudlak syndrome (HPS) is a genetically heterogeneous inherited disease causing hypopigmentation and prolonged bleeding times. An additional serious clinical problem of HPS is the development of lung pathology, which may lead to severe lung disease and premature death. No cure for the disease exists, and previously, no animal model for the HPS lung abnormalities has been reported. A mouse model of HPS, which is homozygously recessive for both the Hps1 (pale ear) and Hps2 (pearl) genes… CONTINUE READING
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  • M Brantly, NA Avila, V Shotelersuk, C Lucero, M Huizing, WA. Gahl
  • AJP-Lung Cell Mol Physiol
  • 2003
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  • S. Hawgood
  • West JB, Weibel ER and Barnes PJ. Philadelphia…
  • 1997
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