Zobrazeno 1 - 10
of 141
pro vyhledávání: '"Bernadette R Gochuico"'
Autor:
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
Publikováno v:
PLoS ONE, Vol 13, Iss 3, p e0194193 (2018)
Pulmonary fibrosis is a progressive, fatal manifestation of Hermansky-Pudlak syndrome (HPS). Some patients with advanced HPS pulmonary fibrosis undergo lung transplantation despite their disease-associated bleeding tendency; others die while awaiting
Externí odkaz:
https://doaj.org/article/f43cfa8e768d44cba8364c5860f58070
Autor:
Kevin White, Yu Lu, Sofia Annis, Andrew E Hale, B Nelson Chau, James E Dahlman, Craig Hemann, Alexander R Opotowsky, Sara O Vargas, Ivan Rosas, Mark A Perrella, Juan C Osorio, Kathleen J Haley, Brian B Graham, Rahul Kumar, Rajan Saggar, Rajeev Saggar, W Dean Wallace, David J Ross, Omar F Khan, Andrew Bader, Bernadette R Gochuico, Majed Matar, Kevin Polach, Nicolai M Johannessen, Haydn M Prosser, Daniel G Anderson, Robert Langer, Jay L Zweier, Laurence A Bindoff, David Systrom, Aaron B Waxman, Richard C Jin, Stephen Y Chan
Publikováno v:
EMBO Molecular Medicine, Vol 7, Iss 6, Pp 695-713 (2015)
Abstract Iron–sulfur (Fe‐S) clusters are essential for mitochondrial metabolism, but their regulation in pulmonary hypertension (PH) remains enigmatic. We demonstrate that alterations of the miR‐210‐ISCU1/2 axis cause Fe‐S deficiencies in v
Externí odkaz:
https://doaj.org/article/2ba2fa97eac5474ab7e2db24f409a724
Autor:
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
Publikováno v:
PLoS ONE, Vol 12, Iss 3, p e0173682 (2017)
Hermansky-Pudlak syndrome (HPS) is a heterogeneous group of genetic disorders typically manifesting with tyrosinase-positive oculocutaneous albinism, bleeding diathesis, and pulmonary fibrosis, in some subtypes. Most HPS subtypes are associated with
Externí odkaz:
https://doaj.org/article/c46a06a58a964271ac86afd5128b04ce
Autor:
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
Publikováno v:
PLoS ONE, Vol 11, Iss 7, p e0159177 (2016)
Hermansky-Pudlak Syndrome type-1 (HPS-1) is an autosomal recessive disorder caused by mutations in HPS1 which result in reduced expression of the HPS-1 protein, defective lysosome-related organelle (LRO) transport and absence of platelet delta granul
Externí odkaz:
https://doaj.org/article/3d18cb7f6c6b42b9b42678afa657559e
Autor:
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
Publikováno v:
PLoS ONE, Vol 9, Iss 5, p e97485 (2014)
OBJECTIVES:Matrix metalloproteinase-8 (MMP-8) promotes lung fibrotic responses to bleomycin in mice. Although prior studies reported that MMP-8 levels are increased in plasma and bronchoalveolar lavage fluid (BALF) samples from IPF patients, neither
Externí odkaz:
https://doaj.org/article/495a074fcbe9498e85c9ca79d471587c
Autor:
Jianmin Xue, Bernadette R Gochuico, Ahmad Samer Alawad, Carol A Feghali-Bostwick, Imre Noth, Steven D Nathan, Glenn D Rosen, Ivan O Rosas, Sanja Dacic, Iclal Ocak, Carl R Fuhrman, Karen T Cuenco, Mary A Smith, Susan S Jacobs, Adriana Zeevi, Penelope A Morel, Joseph M Pilewski, Vincent G Valentine, Kevin F Gibson, Naftali Kaminski, Frank C Sciurba, Yingze Zhang, Steven R Duncan
Publikováno v:
PLoS ONE, Vol 6, Iss 2, p e14715 (2011)
Idiopathic pulmonary fibrosis (IPF) is a progressive and medically refractory lung disease with a grim prognosis. Although the etiology of IPF remains perplexing, abnormal adaptive immune responses are evident in many afflicted patients. We hypothesi
Externí odkaz:
https://doaj.org/article/fd57d3b207be48d59937b9f6e749a878
Autor:
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
Publikováno v:
PLoS Medicine, Vol 5, Iss 4, p e93 (2008)
Idiopathic pulmonary fibrosis (IPF) is a chronic progressive fibrotic lung disease associated with substantial morbidity and mortality. The objective of this study was to determine whether there is a peripheral blood protein signature in IPF and whet
Externí odkaz:
https://doaj.org/article/35e26d851871446c8486502b65b2cf46
Autor:
Muhammad Arif, Abhishek Basu, Kaelin M. Wolf, Joshua K. Park, Lenny Pommerolle, Madeline Behee, Bernadette R. Gochuico, Resat Cinar
Publikováno v:
Advanced Science, Vol 10, Iss 16, Pp n/a-n/a (2023)
Abstract Pulmonary fibrosis (PF) is a heterogeneous disease with a poor prognosis. Therefore, identifying additional therapeutic modalities is required to improve outcome. However, the lack of biomarkers of disease progression hampers the preclinical
Externí odkaz:
https://doaj.org/article/cb60569a0e254ec499c6ba9f6df93296
Autor:
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
Publikováno v:
Respiratory Research, Vol 23, Iss 1, Pp 1-10 (2022)
Abstract Hermansky-Pudlak syndrome (HPS) is an autosomal recessive disorder characterized by improper biogenesis of lysosome-related organelles (LROs). Lung fibrosis is the leading cause of death among adults with HPS-1 and HPS-4 genetic types, which
Externí odkaz:
https://doaj.org/article/7fcff9be0ee44e67a0ff25ba89d3a3c9
Autor:
Karina Cuevas-Mora, Willy Roque, Hoora Shaghaghi, Bernadette R. Gochuico, Ivan O. Rosas, Ross Summer, Freddy Romero
Publikováno v:
Respiratory Research, Vol 22, Iss 1, Pp 1-11 (2021)
Abstract Background Mitochondrial dysfunction has emerged as an important player in the pathogenesis of idiopathic pulmonary fibrosis (IPF), a common cause of idiopathic interstitial lung disease in adults. Hermansky-Pudlak syndrome (HPS) is a rare a
Externí odkaz:
https://doaj.org/article/9baf7f7cb151499797917fd34bd9762a