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Altered lung surfactant system in a Rab38-deficient rat model of Hermansky-Pudlak syndrome.

Osanai K, Higuchi J, Oikawa R, Kobayashi M, Tsuchihara K, Iguchi M, Huang J, Voelker DR, Toga H.

Am J Physiol Lung Cell Mol Physiol. 2010 Feb;298(2):L243-51. doi: 10.1152/ajplung.00242.2009. Epub 2009 Nov 6.


Exogenous gene transfer of Rab38 small GTPase ameliorates aberrant lung surfactant homeostasis in Ruby rats.

Osanai K, Nakase K, Sakuma T, Nishiki K, Nojiri M, Kato R, Saito M, Fujimoto Y, Mizuno S, Toga H.

Respir Res. 2017 Apr 24;18(1):70. doi: 10.1186/s12931-017-0549-2.


Rab38 targets to lamellar bodies and normalizes their sizes in lung alveolar type II epithelial cells.

Zhang L, Yu K, Robert KW, DeBolt KM, Hong N, Tao JQ, Fukuda M, Fisher AB, Huang S.

Am J Physiol Lung Cell Mol Physiol. 2011 Oct;301(4):L461-77. doi: 10.1152/ajplung.00056.2011. Epub 2011 Jul 15.


A mutation in Rab38 small GTPase causes abnormal lung surfactant homeostasis and aberrant alveolar structure in mice.

Osanai K, Oikawa R, Higuchi J, Kobayashi M, Tsuchihara K, Iguchi M, Jongsu H, Toga H, Voelker DR.

Am J Pathol. 2008 Nov;173(5):1265-74. doi: 10.2353/ajpath.2008.080056. Epub 2008 Oct 2.


Analysis and expression of Rab38 in oculocutaneous lung disease.

Osanai K, Voelker DR.

Methods Enzymol. 2008;438:203-15. doi: 10.1016/S0076-6879(07)38014-2.


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

Lyerla TA, Rusiniak ME, Borchers M, Jahreis G, Tan J, Ohtake P, Novak EK, Swank RT.

Am J Physiol Lung Cell Mol Physiol. 2003 Sep;285(3):L643-53. Epub 2003 May 30.


Defective surfactant secretion in a mouse model of Hermansky-Pudlak syndrome.

Guttentag SH, Akhtar A, Tao JQ, Atochina E, Rusiniak ME, Swank RT, Bates SR.

Am J Respir Cell Mol Biol. 2005 Jul;33(1):14-21. Epub 2005 Mar 24.


Expression and localization of a novel Rab small G protein (Rab38) in the rat lung.

Osanai K, Iguchi M, Takahashi K, Nambu Y, Sakuma T, Toga H, Ohya N, Shimizu H, Fisher JH, Voelker DR.

Am J Pathol. 2001 May;158(5):1665-75.


Hermansky Pudlak Syndrome and Pulmonary Alveolar Proteinosis at the same patient: first case report in the world literature.

Ozyilmaz E, Gunasti S, Kuyuku Y, Polat S, Gumurdulu D, Kuleci S, Hanta I, Kocabas A.

Sarcoidosis Vasc Diffuse Lung Dis. 2013 Nov 25;30(3):217-20.


MAP1LC3B overexpression protects against Hermansky-Pudlak syndrome type-1-induced defective autophagy in vitro.

Ahuja S, Knudsen L, Chillappagari S, Henneke I, Ruppert C, Korfei M, Gochuico BR, Bellusci S, Seeger W, Ochs M, Guenther A, Mahavadi P.

Am J Physiol Lung Cell Mol Physiol. 2016 Mar 15;310(6):L519-31. doi: 10.1152/ajplung.00213.2015. Epub 2015 Dec 30.


Impaired Lysosomal Integral Membrane Protein 2-dependent Peroxiredoxin 6 Delivery to Lamellar Bodies Accounts for Altered Alveolar Phospholipid Content in Adaptor Protein-3-deficient pearl Mice.

Kook S, Wang P, Young LR, Schwake M, Saftig P, Weng X, Meng Y, Neculai D, Marks MS, Gonzales L, Beers MF, Guttentag S.

J Biol Chem. 2016 Apr 15;291(16):8414-27. doi: 10.1074/jbc.M116.720201. Epub 2016 Feb 23.


Early alveolar epithelial dysfunction promotes lung inflammation in a mouse model of Hermansky-Pudlak syndrome.

Atochina-Vasserman EN, Bates SR, Zhang P, Abramova H, Zhang Z, Gonzales L, Tao JQ, Gochuico BR, Gahl W, Guo CJ, Gow AJ, Beers MF, Guttentag S.

Am J Respir Crit Care Med. 2011 Aug 15;184(4):449-58. doi: 10.1164/rccm.201011-1882OC.


Epithelial stress and apoptosis underlie Hermansky-Pudlak syndrome-associated interstitial pneumonia.

Mahavadi P, Korfei M, Henneke I, Liebisch G, Schmitz G, Gochuico BR, Markart P, Bellusci S, Seeger W, Ruppert C, Guenther A.

Am J Respir Crit Care Med. 2010 Jul 15;182(2):207-19. doi: 10.1164/rccm.200909-1414OC. Epub 2010 Apr 8.


The cell biology of Hermansky-Pudlak syndrome: recent advances.

Di Pietro SM, Dell'Angelica EC.

Traffic. 2005 Jul;6(7):525-33. Review.


Histochemical and cellular changes accompanying the appearance of lung fibrosis in an experimental mouse model for Hermansky Pudlak syndrome.

Wang L, Lyerla T.

Histochem Cell Biol. 2010 Aug;134(2):205-13. doi: 10.1007/s00418-010-0724-8. Epub 2010 Jul 7.


Analysis of ocular hypopigmentation in Rab38cht/cht mice.

Brooks BP, Larson DM, Chan CC, Kjellstrom S, Smith RS, Crawford MA, Lamoreux L, Huizing M, Hess R, Jiao X, Hejtmancik JF, Maminishkis A, John SW, Bush R, Pavan WJ.

Invest Ophthalmol Vis Sci. 2007 Sep;48(9):3905-13.


Vacuolar ATPase regulates surfactant secretion in rat alveolar type II cells by modulating lamellar body calcium.

Chintagari NR, Mishra A, Su L, Wang Y, Ayalew S, Hartson SD, Liu L.

PLoS One. 2010 Feb 16;5(2):e9228. doi: 10.1371/journal.pone.0009228.


[Effects of erythromycin on surfactant synthesis, secretion, and recycling by rat alveolar type II cells].

Suwabe A, Ito M, Takahashi K.

Nihon Kyobu Shikkan Gakkai Zasshi. 1996 Oct;34(10):1104-8. Japanese.


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