Malfolded Protein Structure and Proteostasis in Lung Diseases

被引：52

作者：

Balch, William E. ^{[1
]}

Sznajder, Jacob I. ^{[2
]}

Budinger, Scott ^{[2
]}

Finley, Daniel ^{[4
]}

Laposky, Aaron D. ^{[5
]}

Cuervo, Ana Maria ^{[6
,7
]}

Benjamin, Ivor J. ^{[8
]}

Barreiro, Esther ^{[9
]}

Morimoto, Richard I. ^{[3
]}

Postow, Lisa ^{[5
]}

Weissman, Allan M. ^{[10
,11
]}

Gail, Dorothy ^{[5
]}

Banks-Schlegel, Susan ^{[5
]}

Croxton, Thomas ^{[5
]}

Gan, Weiniu ^{[5
]}

机构：

[1] Scripps Res Inst, Dept Cell Biol & Chem Physiol, La Jolla, CA 92037 USA

[2] Northwestern Univ, Div Pulm & Crit Care Med, Feinberg Sch Med, Chicago, IL 60611 USA

[3] Northwestern Univ, Dept Mol Biosci, Chicago, IL 60611 USA

[4] Harvard Univ, Sch Med, Dept Cell Biol, Boston, MA USA

[5] NHLBI, Div Lung Dis, NIH, Bethesda, MD 20892 USA

[6] Albert Einstein Coll Med, Dept Dev & Mol Biol, Bronx, NY 10467 USA

[7] Albert Einstein Coll Med, Inst Aging Studies, Bronx, NY 10467 USA

[8] Med Coll Wisconsin, Div Cardiovasc Med, Milwaukee, WI 53226 USA

[9] Hosp del Mar, Dept Resp Med, Lung Canc Res Grp, Inst Med Res Hosp del Mar IMIM,CIBERES, Barcelona, Spain

[10] NCI, Lab Prot Dynam, Frederick, MD 21701 USA

[11] NCI, Signaling Ctr Canc Res, Frederick, MD 21701 USA

来源：

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE | 2014年 / 189卷 / 01期

关键词：

protein misfolding; post-translational processing; proteosome; ubiquitination; pulmonary health; OBSTRUCTIVE PULMONARY-DISEASE; ENDOPLASMIC-RETICULUM STRESS; OXIDATIVE STRESS; THERAPEUTIC PARADIGM; FIBROSIS; AUTOPHAGY; MUC5B; DEGRADATION; PROTEASOME; STRATEGIES;

D O I：

10.1164/rccm.201306-1164WS

中图分类号：

R4 [临床医学];

学科分类号：

1002 ; 100602 ;

摘要：

Recent discoveries indicate that disorders of protein folding and degradation play a particularly important role in the development of lung diseases and their associated complications. The overarching purpose of the National Heart, Lung, and Blood Institute workshop on "Malformed Protein Structure and Proteostasis in Lung Diseases" was to identify mechanistic and clinical research opportunities indicated by these recent discoveries in proteostasis science that will advance our molecular understanding of lung pathobiology and facilitate the development of new diagnostic and therapeutic strategies for the prevention and treatment of lung disease. The workshop's discussion focused on identifying gaps in scientific knowledge with respect to proteostasis and lung disease, discussing new research advances and opportunities in protein folding science, and highlighting novel technologies with potential therapeutic applications for diagnosis and treatment.

引用

页码：96 / 103

页数：8

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