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1

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

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

Journal:

Orphanet Journal of Rare Diseases

Year:

2019

Language:

english

File:

PDF, 2.14 MB

english, 2019

2

Hermansky‐Pudlak Syndrome: Mutation Update

Huizing, Marjan, Malicdan, May Christine V., Wang, Jennifer A., Pri‐Chen, Hadass, Hess, Richard A., Fischer, Roxanne, O'Brien, Kevin J., Merideth, Melissa A., Gahl, William A., Gochuico, Bernadette R.

Journal:

Year:

2020

Language:

english

File:

PDF, 1.15 MB

english, 2020

3

Characterization and peripheral blood biomarker assessment of anti–Jo-1 antibody–positive interstitial lung disease

Thomas J. Richards, Aaron Eggebeen, Kevin Gibson, Samuel Yousem, Carl Fuhrman, Bernadette R. Gochuico, Noreen Fertig, Chester V. Oddis, Naftali Kaminski, Ivan O. Rosas, Dana P. Ascherman

Journal:

Arthritis & Rheumatism

Year:

2009

Language:

english

File:

PDF, 281 KB

english, 2009

4

Prevalence of uterine leiomyomas in lymphangioleiomyomatosis

Angelo M. Taveira-DaSilva, Antoinette Rabel, Bernadette R. Gochuico, Nilo A. Avila, Joel Moss

Journal:

Fertility and Sterility

Year:

2011

Language:

english

File:

PDF, 193 KB

english, 2011

5

Pirfenidone for the treatment of Hermansky–Pudlak syndrome pulmonary fibrosis

Kevin O'Brien, James Troendle, Bernadette R. Gochuico, Thomas C. Markello, Jose Salas, Hilda Cardona, Jianhua Yao, Isa Bernardini, Richard Hess, William A. Gahl

Journal:

Molecular Genetics and Metabolism

Year:

2011

Language:

english

File:

PDF, 667 KB

english, 2011

6

Effect of pirfenidone on the pulmonary fibrosis of Hermansky–Pudlak syndrome

William A Gahl, Mark Brantly, James Troendle, Nilo A Avila, Antonio Padua, Carlos Montalvo, Hilda Cardona, Karim Anton Calis, Bernadette Gochuico

Journal:

Molecular Genetics and Metabolism

Year:

2002

Language:

english

File:

PDF, 238 KB

english, 2002

7

Identification of Citrullinated Hsp90 Isoforms as Novel Autoantigens in Rheumatoid Arthritis-Associated Interstitial Lung Disease

Harlow, Lisa, Rosas, Ivan O., Gochuico, Bernadette R., Mikuls, Ted R., Dellaripa, Paul F., Oddis, Chester V., Ascherman, Dana P.

Journal:

Arthritis & Rheumatism

Year:

2013

Language:

english

File:

PDF, 240 KB

english, 2013

8

Glucose Transporter-1 Distribution in Fibrotic Lung Disease

El-Chemaly, Souheil, Malide, Daniela, Yao, Jianhua, Nathan, Steven D., Rosas, Ivan O., Gahl, William A., Moss, Joel, Gochuico, Bernadette R.

Journal:

Year:

2013

Language:

english

File:

PDF, 1.48 MB

english, 2013

9

Syndecan-2 Exerts Antifibrotic Effects by Promoting Caveolin-1–mediated Transforming Growth Factor-β Receptor I Internalization and Inhibiting Transforming Growth Factor-β1 Signaling

Shi, Yuanyuan, Gochuico, Bernadette R., Yu, Guoying, Tang, Xiaomeng, Osorio, Juan C., Fernandez, Isis E., Risquez, Cristobal F., Patel, Avignat S., Shi, Ying, Wathelet, Marc G., Goodwin, Andrew J., Ha

Journal:

American Journal of Respiratory and Critical Care Medicine

Year:

2013

Language:

english

File:

PDF, 10.57 MB

english, 2013

10

Anti-citrullinated heat shock protein 90 antibodies identified in bronchoalveolar lavage fluid are a marker of lung-specific immune responses

Harlow, Lisa, Gochuico, Bernadette R., Rosas, Ivan O., Doyle, Tracy J., Osorio, Juan C., Travers, Timothy S., Camacho, Carlos C., Oddis, Chester V., Ascherman, Dana P.

Journal:

Clinical Immunology

Year:

2014

Language:

english

File:

PDF, 1.51 MB

english, 2014

11

Circulating Fibrocytes as Biomarker of Prognosis in Hermansky-Pudlak Syndrome

Trimble, Aaron, Gochuico, Bernadette R., Markello, Thomas C., Fischer, Roxanne, Gahl, William A., Lee, Jae K., Kim, Youngchul, Burdick, Marie D., Strieter, Robert M., Mehrad, Borna

Journal:

American Journal of Respiratory and Critical Care Medicine

Year:

2014

Language:

english

File:

PDF, 1.01 MB

english, 2014

12

In vitro functional correction of Hermansky–Pudlak Syndrome type-1 by lentiviral-mediated gene transfer

Ikawa, Yasuhiro, Hess, Richard, Dorward, Heidi, Cullinane, Andrew R., Huizing, Marjan, Gochuico, Bernadette R., Gahl, William A., Candotti, Fabio

Journal:

Molecular Genetics and Metabolism

Year:

2015

Language:

english

File:

PDF, 685 KB

english, 2015

13

Nuclear Localization of Vascular Endothelial Growth Factor-D and Regulation of c-Myc–Dependent Transcripts in Human Lung Fibroblasts

El-Chemaly, Souheil, Pacheco-Rodriguez, Gustavo, Malide, Daniela, Meza-Carmen, Victor, Kato, Jiro, Cui, Ye, Padilla, Philip I., Samidurai, Arun, Gochuico, Bernadette R., Moss, Joel

Journal:

American Journal of Respiratory Cell and Molecular Biology

Year:

2014

Language:

english

File:

PDF, 1.94 MB

english, 2014

14

Progressive Preclinical Interstitial Lung Disease in Rheumatoid Arthritis

Gochuico, Bernadette R.

Journal:

Archives of Internal Medicine

Year:

2008

Language:

english

File:

PDF, 201 KB

english, 2008

15

Clinical, Molecular, and Cellular Features of Non-Puerto Rican Hermansky–Pudlak Syndrome Patients of Hispanic Descent

Carmona-Rivera, Carmelo, Golas, Gretchen, Hess, Richard A, Cardillo, Nicholas D, Martin, Elijah H, O'Brien, Kevin, Tsilou, Ekaterini, Gochuico, Bernadette R, White, James G, Huizing, Marjan, Gahl, Wil

Journal:

The Journal of Investigative Dermatology

Year:

2011

Language:

english

File:

PDF, 1.74 MB

english, 2011

16

Alveolar Macrophage Dysregulation in Hermansky-Pudlak Syndrome Type 1

Rouhani, Farshid N., Brantly, Mark L., Markello, Thomas C., Helip-Wooley, Amanda, O'Brien, Kevin, Hess, Richard, Huizing, Marjan, Gahl, William A., Gochuico, Bernadette R.

Journal:

American Journal of Respiratory and Critical Care Medicine

Year:

2009

Language:

english

File:

PDF, 357 KB

english, 2009

17

Epithelial Stress and Apoptosis Underlie Hermansky-Pudlak Syndrome–associated Interstitial Pneumonia

Mahavadi, Poornima, Korfei, Martina, Henneke, Ingrid, Liebisch, Gerhard, Schmitz, Gerd, Gochuico, Bernadette R., Markart, Philipp, Bellusci, Saverio, Seeger, Werner, Ruppert, Clemens, Guenther, Andrea

Journal:

American Journal of Respiratory and Critical Care Medicine

Year:

2010

Language:

english

File:

PDF, 3.92 MB

english, 2010

18

Hermansky-Pudlak Syndrome Interstitial Pneumonia

Mahavadi, Poornima, Guenther, Andreas, Gochuico, Bernadette R.

Journal:

American Journal of Respiratory and Critical Care Medicine

Year:

2012

Language:

english

File:

PDF, 53 KB

english, 2012

19

Impairment of Alveolar Macrophage Transcription in Idiopathic Pulmonary Fibrosis

Ren, Ping, Rosas, Ivan O., MacDonald, Sandra D., Wu, Hai-Ping, Billings, Eric M., Gochuico, Bernadette R.

Journal:

American Journal of Respiratory and Critical Care Medicine

Year:

2007

Language:

english

File:

PDF, 1.27 MB

english, 2007

20

Early Alveolar Epithelial Dysfunction Promotes Lung Inflammation in a Mouse Model of Hermansky-Pudlak Syndrome

Atochina-Vasserman, Elena N., Bates, Sandra R., Zhang, Peggy, Abramova, Helen, Zhang, Zhenguo, Gonzales, Linda, Tao, Jian-Qin, Gochuico, Bernadette R., Gahl, William, Guo, Chang-Jiang, Gow, Andrew J.,

Journal:

American Journal of Respiratory and Critical Care Medicine

Year:

2011

Language:

english

File:

PDF, 1.14 MB

english, 2011

21

Automated Quantification of High-Resolution CT Scan Findings in Individuals at Risk for Pulmonary Fibrosis

Rosas, Ivan O., Yao, Jianhua, Avila, Nilo A., Chow, Catherine K., Gahl, William A., Gochuico, Bernadette R.

Journal:

Year:

2011

Language:

english

File:

PDF, 773 KB

english, 2011

22

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

Ahuja, Saket, Knudsen, Lars, Chillappagari, Shashi, Henneke, Ingrid, Ruppert, Clemens, Korfei, Martina, Gochuico, Bernadette R., Bellusci, Saverio, Seeger, Werner, Ochs, Matthias, Guenther, Andreas, M

Journal:

AJP Lung Cellular and Molecular Physiology

Year:

2016

Language:

english

File:

PDF, 4.52 MB

english, 2016

23

Pulmonary Dysfunction in Adults With Nephropathic Cystinosis

Anikster, Yair, Lacbawan, Felicitas, Brantly, Mark, Gochuico, Bernadette L., Avila, Nilo A., Travis, William, Gahl, William A.

Journal:

Year:

2001

Language:

english

File:

PDF, 1.88 MB

english, 2001

24

Potential Pathogenesis and Clinical Aspects of Pulmonary Fibrosis Associated with Rheumatoid Arthritis

Gochuico, Bernadette R.

Journal:

The American Journal of the Medical Sciences

Year:

2001

Language:

english

File:

PDF, 116 KB

english, 2001

25

Elevation in Peripheral Fibrocyte Counts Correlates With Presence of Interstitial Lung Disease in Patients With Hermansky-Pudlak Syndrome

Trimble, Aaron, Burdick, Marie, Fischer, Roxanne, Gahl, William, Gochuico, Bernadette, Mehrad, Borna

Journal:

Year:

2013

Language:

english

File:

PDF, 187 KB

english, 2013

26

Natural History of Pulmonary Fibrosis in Two Subjects With the Same Telomerase Mutation

El-Chemaly, Souheil, Ziegler, Shira G., Calado, Rodrigo T., Wilson, Kirkland A., Wu, Hai Ping, Haughey, Mary, Peterson, Nathan R., Young, Neal S., Gahl, William A., Moss, Joel, Gochuico, Bernadette R.

Journal:

Year:

2011

Language:

english

File:

PDF, 1.30 MB

english, 2011

27

PIRFENIDONE FOR TREATMENT OF PULMONARY FIBROSIS IN HERMANSKY-PUDLAK SYNDROME-1

Gochuico, Bernadette R., Markello, Thomas C., O'Brien, Kevin J., Cardona, Hilda, Salas, Jose, Gahl, William A.

Journal:

Year:

2008

Language:

english

File:

PDF, 154 KB

english, 2008

28

[American Thoracic Society American Thoracic Society 2011 International Conference, May 13-18, 2011 • Denver Colorado - ()] C109. 'OMICS APPROACHES TO LUNG DISEASES - Epigenetic Mechanisms Modulate TGF-B Induced PAI-1 Expression In Pulmonary Fibrosis

McBrine, Michael, Fitzgibbons, CM, Vo, T, Lakshminarayanan, M, Gochuico, Bernadette R., Cao, Y, Ramirez, Maria I

Year:

2011

Language:

english

File:

PDF, 17 KB

english, 2011

29

[American Thoracic Society American Thoracic Society 2010 International Conference, May 14-19, 2010 • New Orleans - ()] D48. INTERSTITIAL LUNG DISEASE: PATHOBIOLOGY, BIOMARKERS AND TREATMENT - Interstitial Lung Disease In Hermansky-Pudlak Syndrome-2

Gochuico, Bernadette R., Huizing, Marjan, Golas, Gretchen, Gahl, William A.

Year:

2010

Language:

english

File:

PDF, 17 KB

english, 2010

30

SPIE Proceedings [SPIE SPIE Medical Imaging - Lake Buena Vista, Florida (Saturday 12 February 2011)] Medical Imaging 2011: Computer-Aided Diagnosis - Enhancing image classification models with multi-modal biomarkers

Caban, Jesus J., Summers, Ronald M., van Ginneken, Bram, Liao, David, Yao, Jianhua, Mollura, Daniel J., Gochuico, Bernadette, Yoo, Terry

Year:

2011

Language:

english

File:

PDF, 1.08 MB

english, 2011

31

Peribronchiolar Metaplasia: A Common Histologic Lesion in Diffuse Lung Disease and a Rare Cause of Interstitial Lung Disease

Fukuoka, Junya, Franks, Teri J, Colby, Thomas V, Flaherty, Kevin R, Galvin, Jeffrey R, Hayden, Dennis, Gochuico, Bernadette R, Kazerooni, Ella A, Martinez, Fernando, Travis, William D

Journal:

American Journal of Surgical Pathology

Year:

2005

Language:

english

File:

PDF, 670 KB

english, 2005

32

Natural killer cell activity and dysfunction in Hermansky-Pudlak syndrome

Gil-Krzewska, Aleksandra, Murakami, Yousuke, Peruzzi, Giovanna, O'Brien, Kevin J., Merideth, Melissa A., Cullinane, Andrew R., Gahl, William A., Coligan, John E., Gochuico, Bernadette R., Krzewski, Ko

Journal:

British Journal of Haematology

Year:

2016

Language:

english

File:

PDF, 511 KB

english, 2016

33

A YAP/TAZ-miR-130/301 molecular circuit exerts systems-level control of fibrosis in a network of human diseases and physiologic conditions

Bertero, Thomas, Cottrill, Katherine A., Annis, Sofia, Bhat, Balkrishen, Gochuico, Bernadette R., Osorio, Juan C., Rosas, Ivan, Haley, Kathleen J., Corey, Kathleen E., Chung, Raymond T., Nelson Chau,

Journal:

Scientific Reports

Year:

2015

Language:

english

File:

PDF, 5.37 MB

english, 2015

34

Usual Interstitial Pneumonitis Responsive to Corticosteroids Following Varicella Pneumonia

Keane, Joseph, Gochuico, Bernadette, Kasznica, John M., Kornfeld, Hardy

Journal:

Year:

1998

Language:

english

File:

PDF, 2.21 MB

english, 1998

35

CA-125 in Disease Progression and Treatment of Lymphangioleiomyomatosis

Glasgow, Connie G., Pacheco-Rodriguez, Gustavo, Steagall, Wendy K., Haughey, Mary E., Julien-Williams, Patricia A., Stylianou, Mario P., Gochuico, Bernadette R., Moss, Joel

Journal:

Year:

2017

Language:

english

File:

PDF, 2.09 MB

english, 2017

36

Erdheim-Chester Disease: A Rare Multisystem Histiocytic Disorder Associated with Interstitial Lung Disease

Shamburek, Robert D., Brewer, H. Bryan, Gochuico, Bernadette R.

Journal:

The American Journal of the Medical Sciences

Year:

2001

Language:

english

File:

PDF, 1.43 MB

english, 2001

37

The curse of idiopathic

O’Brien, Kevin J., Gahl, William A., Gochuico, Bernadette R.

Journal:

Journal of Inherited Metabolic Disease

Year:

2017

Language:

english

File:

PDF, 286 KB

english, 2017

38

Matrix Metalloproteinase Activity is Increased in the Lungs in Hermansky-Pudlak Syndrome

Krishna, Rachana, Shriner, Deleila, Gochuico, Bernadette, Summer, Ross, Romero, Freddy

Journal:

Year:

2017

Language:

english

File:

PDF, 67 KB

english, 2017

39

Galectin-3 Interacts with the CHI3L1 Axis and Contributes to Hermansky–Pudlak Syndrome Lung Disease

Zhou, Yang, He, Chuan Hua, Yang, Daniel S., Nguyen, Tung, Cao, Yueming, Kamle, Suchitra, Lee, Chang-min, Gochuico, Bernadette R., Gahl, William A., Shea, Barry S., Lee, Chun Geun, Elias, Jack A.

Journal:

The Journal of Immunology

Year:

2018

Language:

english

File:

PDF, 2.24 MB

english, 2018

40

Mononuclear Phagocytes and Airway Epithelial Cells: Novel Sources of Matrix Metalloproteinase-8 (MMP-8) in Patients with Idiopathic Pulmonary Fibrosis

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

Journal:

Year:

2014

Language:

english

File:

PDF, 2.50 MB

english, 2014

41

The Immunome in Two Inherited Forms of Pulmonary Fibrosis

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

Journal:

Frontiers in Immunology

Year:

2018

Language:

english

File:

PDF, 4.73 MB

english, 2018

42

Prolonged treatment with open-label pirfenidone in Hermansky-Pudlak syndrome pulmonary fibrosis

O'Brien, Kevin J., Introne, Wendy J., Akal, Orhan, Akal, Tulay, Barbu, Adrian, McGowan, Melissa P., Merideth, Melissa A., Seward, Samuel L., Gahl, William A., Gochuico, Bernadette R.

Journal:

Molecular Genetics and Metabolism

Year:

2018

Language:

english

File:

PDF, 1.20 MB

english, 2018

43

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

Haroutunian, Sara, O’Brien, Kevin, Estrada-Veras, Juvianee, Yao, Jianhua, Boyd, Louisa, Mathur, Kavya, Gahl, William, Mirmomen, S., Malayeri, Ashkan, Kleiner, David, Jaffe, Elaine, Gochuico, Bernadett

Journal:

Journal of Clinical Medicine

Year:

2018

Language:

english

File:

PDF, 1.31 MB

english, 2018

44

Expanding the phenotype of COPA syndrome: a kindred with typical and atypical features

Taveira-DaSilva, Angelo M, Markello, Thomas C, Kleiner, David E, Jones, Amanda M, Groden, Catherine, Macnamara, Ellen, Yokoyama, Tadafumi, Gahl, William A, Gochuico, Bernadette R, Moss, Joel

Journal:

Journal of Medical Genetics

Year:

2018

Language:

english

File:

PDF, 842 KB

english, 2018

45

Transforming Growth Factor-β1 Downregulates Vascular Endothelial Growth Factor-D Expression in Human Lung Fibroblasts via the Jun NH2-Terminal Kinase Signaling Pathway

Cui, Ye, Osorio, Juan C., Risquez, Cristobal, Wang, Hao, Shi, Ying, Gochuico, Bernadette R., Morse, Danielle, Rosas, Ivan O., El-Chemaly, Souheil

Journal:

Molecular Medicine

Year:

2014

File:

PDF, 5.84 MB

2014

46

Bleomycin Induces Drug Efflux in Lungs: A pitfall for Pharmacological Studies of Pulmonary Fibrosis

Park, Joshua K., Coffey, Nathan J, Bodine, Steven P., Zawatsky, Charles N, Jay, Lindsey, Gahl, William A, Kunos, George, Gochuico, Bernadette R, Malicdan, May Christine V., Cinar, Resat

Journal:

American Journal of Respiratory Cell and Molecular Biology

Year:

2019

File:

PDF, 1.52 MB

2019

47

Genetic variants associated with Hermansky-Pudlak syndrome

Merideth, Melissa A., Introne, Wendy J., Wang, Jennifer A., O’Brien, Kevin J., Huizing, Marjan, Gochuico, Bernadette R.

Journal:

Year:

2019

Language:

english

File:

PDF, 719 KB

english, 2019

48

A comprehensive, multidisciplinary, precision medicine approach to discover effective therapy for an undiagnosed, progressive, fibroinflammatory disease

Gochuico, Bernadette R., Ziegler, Shira G., Ten, Nicholas S., Balanda, Nicholas J., Mason, Christopher E., Zumbo, Paul, Evans, Colleen A., Van Waes, Carter, Gahl, William A., Malicdan, May C.V.

Journal:

Translational Research

Year:

2019

Language:

english

File:

PDF, 2.89 MB

english, 2019

49

Hermansky‐Pudlak Syndrome: Mutation Update

Huizing, Marjan, Malicdan, May Christine V., Wang, Jennifer A., Pri‐Chen, Hadass, Hess, Richard A., Fischer, Roxanne, O'Brien, Kevin J., Merideth, Melissa A., Gahl, William A., Gochuico, Bernadette R.

Journal:

Year:

2020

File:

PDF, 1.15 MB

2020

50

[American Thoracic Society American Thoracic Society 2012 International Conference, May 18-23, 2012 • San Francisco, California - ()] B66. THE FIBROBLAST POOL ON THE MOVE: DERIVATION AND TARGETING - Galectin-3 Co-Localizes With EEA-1 In Type II Cells In Hermansky-Pudlak Syndrome Pulmonary Fibrosis

Gochuico, Bernadette R., Dorward, Heidi, Yeager, Caroline, Gomez, Blanca J., Gahl, William A.

Year:

2012

File:

PDF, 16 KB

2012

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