4-Phenylbutyric Acid Treatment Rescues Trafficking and Processing of a Mutant Surfactant Protein–C

Amyloid-like Aggregates Sequester Numerous Metastable Proteins with Essential Cellular Functions

(2011) Heidi Olzscha et al.   CELL

Interstitial lung disease in children

(2011) Shailendra Das et al.   CURRENT OPINION IN PEDIATRICS

BRICHOS domain associated with lung fibrosis, dementia and cancer - a chaperone that prevents amyloid fibril formation?

(2011) Hanna Willander et al.   FEBS Journal

Azithromycin blocks autophagy and may predispose cystic fibrosis patients to mycobacterial infection

(2011) Maurizio Renna et al.   JOURNAL OF CLINICAL INVESTIGATION

Idiopathic pulmonary fibrosis

(2011) Talmadge E King et al.   LANCET

Expression and In Vivo Rescue of Human ABCC6 Disease-Causing Mutants in Mouse Liver

(2011) Olivier Le Saux et al.   PLoS One

A Nonaggregating Surfactant Protein C Mutant Is Misdirected to Early Endosomes and Disrupts Phospholipid Recycling

(2011) Michael F. Beers et al.   TRAFFIC

Surfactant Protein C Mutations Are the Basis of a Significant Portion of Adult Familial Pulmonary Fibrosis in a Dutch Cohort

(2010) Coline H. M. van Moorsel et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Amino acid sequence determinants and molecular chaperones in amyloid fibril formation

(2010) Charlotte Nerelius et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Restoring endoplasmic reticulum function by chemical chaperones: an emerging therapeutic approach for metabolic diseases

(2010) F. Engin et al.   DIABETES OBESITY & METABOLISM

Surfactant protein C gene (SFTPC) mutation-associated lung disease: High-resolution computed tomography (HRCT) findings and its relation to histological analysis

(2010) M. Mechri et al.   PEDIATRIC PULMONOLOGY

Nedd4-2–Mediated Ubiquitination Facilitates Processing of Surfactant Protein–C

(2009) Juliana J. Conkright et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

The Brichos domain of prosurfactant protein C can hold and fold a transmembrane segment

(2009) Hanna Johansson et al.   PROTEIN SCIENCE

Mutations linked to interstitial lung disease can abrogate anti-amyloid function of prosurfactant protein C

(2008) Charlotte Nerelius et al.   BIOCHEMICAL JOURNAL

ERdj4 and ERdj5 Are Required for Endoplasmic Reticulum-associated Protein Degradation of Misfolded Surfactant Protein C

(2008) Mei Dong et al.   MOLECULAR BIOLOGY OF THE CELL