Hermansky–Pudlak syndrome pulmonary fibrosis: a rare inherited interstitial lung disease

by Tadafumi Yokoyama, Bernadette R. Gochuico
Published 2021-02-01

Hermansky-Pudlak syndrome-2 alters mitochondrial homeostasis in the alveolar epithelium of the lung

by Karina Cuevas-Mora, Willy Roque, Hoora Shaghaghi, Bernadette R. Gochuico, Ivan O. Rosas, Ross Summer, Freddy Romero
Published 2021-02-01

Genetic variants associated with Hermansky-Pudlak syndrome

by Melissa A. Merideth, Wendy J. Introne, Jennifer A. Wang, Kevin J. O’Brien, Marjan Huizing, Bernadette R. Gochuico
Published 2020-05-01

An Integrative Multiomics Framework for Identification of Therapeutic Targets in Pulmonary Fibrosis

by Muhammad Arif, Abhishek Basu, Kaelin M. Wolf, Joshua K. Park, Lenny Pommerolle, Madeline Behee, Bernadette R. Gochuico, Resat Cinar
Published 2023-06-01

Quantification of Proteus syndrome-associated lung disease

by Christopher A. Ours, Anna Buser, Mia B. Hodges, Marcus Y. Chen, Julie C. Sapp, Bernadette R. Gochuico, Leslie G. Biesecker
Published 2024-02-01

Matrix metalloproteinase activity in the lung is increased in Hermansky-Pudlak syndrome

by Ross Summer, Rachana Krishna, DeLeila Schriner, Karina Cuevas-Mora, Dominic Sales, Rachel Para, Jesse Roman, Carl Nieweld, Bernadette R. Gochuico, Freddy Romero
Published 2019-07-01

The Immunome in Two Inherited Forms of Pulmonary Fibrosis

by Souheil El-Chemaly, Foo Cheung, Yuri Kotliarov, Kevin J. O’Brien, William A. Gahl, William A. Gahl, Jinguo Chen, Shira Y. Perl, Angélique Biancotto, Bernadette R. Gochuico
Published 2018-01-01

Mononuclear phagocytes and airway epithelial cells: novel sources of matrix metalloproteinase-8 (MMP-8) in patients with idiopathic pulmonary fibrosis.

by Vanessa J Craig, Francesca Polverino, Maria E Laucho-Contreras, Yuanyuan Shi, Yushi Liu, Juan C Osorio, Yohannes Tesfaigzi, Victor Pinto-Plata, Bernadette R Gochuico, Ivan O Rosas, Caroline A Owen
Published 2014-01-01

Immunophenotypic and Ultrastructural Analysis of Mast Cells in Hermansky-Pudlak Syndrome Type-1: A Possible Connection to Pulmonary Fibrosis.

by Arnold S Kirshenbaum, Glenn Cruse, Avanti Desai, Geethani Bandara, Maarten Leerkes, Chyi-Chia R Lee, Elizabeth R Fischer, Kevin J O'Brien, Bernadette R Gochuico, Kelly Stone, William A Gahl, Dean D Metcalfe
Published 2016-01-01

Neurological manifestations of Erdheim–Chester Disease

by Louisa C. Boyd, Kevin J. O’Brien, Neval Ozkaya, Tanya Lehky, Avner Meoded, Bernadette R. Gochuico, Fady Hannah‐Shmouni, Avindra Nath, Camilo Toro, William A. Gahl, Juvianee I. Estrada‐Veras, Rahul H. Dave
Published 2020-04-01

Hermansky-Pudlak syndrome and oculocutaneous albinism in Chinese children with pigmentation defects and easy bruising

by Bradley Power, Carlos R. Ferreira, Dong Chen, Wadih M. Zein, Kevin J. O’Brien, Wendy J. Introne, Joshi Stephen, William A. Gahl, Marjan Huizing, May Christine V. Malicdan, David R. Adams, Bernadette R. Gochuico
Published 2019-02-01

Clinical and Histopathologic Features of Interstitial Lung Disease in Erdheim–Chester Disease

by Sara G. Haroutunian, Kevin J. O’Brien, Juvianee I. Estrada-Veras, Jianhua Yao, Louisa C. Boyd, Kavya Mathur, William A. Gahl, S. Mojdeh Mirmomen, Ashkan A. Malayeri, David E. Kleiner, Elaine S. Jaffe, Bernadette R. Gochuico
Published 2018-08-01

Pituitary Imaging Abnormalities and Related Endocrine Disorders in Erdheim–Chester Disease

by Skand Shekhar, Jorge A. Irizarry-Caro, Ninet Sinaii, William A. Gahl, Juvianee I. Estrada-Veras, Rahul H. Dave, Bernadette R. Gochuico, Georgios Z. Papadakis, Nicholas Patronas, Constantine A. Stratakis, Kevin O’Brien, Fady Hannah-Shmouni
Published 2021-08-01

MMP1 and MMP7 as potential peripheral blood biomarkers in idiopathic pulmonary fibrosis.

by Ivan O Rosas, Thomas J Richards, Kazuhisa Konishi, Yingze Zhang, Kevin Gibson, Anna E Lokshin, Kathleen O Lindell, Jose Cisneros, Sandra D Macdonald, Annie Pardo, Frank Sciurba, James Dauber, Moises Selman, Bernadette R Gochuico, Naftali Kaminski
Published 2008-04-01

Automated Digital Quantification of Pulmonary Fibrosis in Human Histopathology Specimens

by Lauren C. Testa, Yvon Jule, Linnea Lundh, Karine Bertotti, Melissa A. Merideth, Kevin J. O'Brien, Steven D. Nathan, Drew C. Venuto, Souheil El-Chemaly, May Christine V. Malicdan, May Christine V. Malicdan, Bernadette R. Gochuico
Published 2021-06-01

Cellular and molecular defects in a patient with Hermansky-Pudlak syndrome type 5.

by Joshi Stephen, Tadafumi Yokoyama, Nathanial J Tolman, Kevin J O'Brien, Elena-Raluca Nicoli, Brian P Brooks, Laryssa Huryn, Steven A Titus, David R Adams, Dong Chen, William A Gahl, Bernadette R Gochuico, May Christine V Malicdan
Published 2017-01-01

Progressive pulmonary fibrosis in a murine model of Hermansky-Pudlak syndrome

by Shachar Abudi-Sinreich, Steven P. Bodine, Tadafumi Yokoyama, Nathanial J. Tolman, Michal Tyrlik, Lauren C. Testa, Chen G. Han, Heidi M. Dorward, Stephen M. Wincovitch, Yair Anikster, William A. Gahl, Resat Cinar, Bernadette R. Gochuico, May Christine V. Malicdan
Published 2022-05-01

Clinical management and outcomes of patients with Hermansky-Pudlak syndrome pulmonary fibrosis evaluated for lung transplantation.

by Souheil El-Chemaly, Kevin J O'Brien, Steven D Nathan, Gerald L Weinhouse, Hilary J Goldberg, Jean M Connors, Ye Cui, Todd L Astor, Philip C Camp, Ivan O Rosas, Merte Lemma, Vladislav Speransky, Melissa A Merideth, William A Gahl, Bernadette R Gochuico
Published 2018-01-01

Dysregulated myosin in Hermansky-Pudlak syndrome lung fibroblasts is associated with increased cell motility

by Jewel Imani, Steven P. M. Bodine, Anthony M. Lamattina, Diane D. Ma, Shikshya Shrestha, Dawn M. Maynard, Kevin Bishop, Arinze Nwokeji, May Christine V. Malicdan, Lauren C. Testa, Raman Sood, Benjamin Stump, Ivan O. Rosas, Mark A. Perrella, Robert Handin, Lisa R. Young, Bernadette R. Gochuico, Souheil El-Chemaly
Published 2022-06-01

CB1R and iNOS are distinct players promoting pulmonary fibrosis in Hermansky–Pudlak syndrome

by Resat Cinar, Joshua K. Park, Charles N. Zawatsky, Nathan J. Coffey, Steven P. Bodine, Jasmina Abdalla, Tadafumi Yokoyama, Tony Jourdan, Lindsey Jay, Mei Xing G. Zuo, Kevin J. O'Brien, Junfeng Huang, Ken Mackie, Asaf Alimardanov, Malliga R. Iyer, William A. Gahl, George Kunos, Bernadette R. Gochuico, May Christine V. Malicdan
Published 2021-07-01