A non-BRICHOS SFTPC mutant (SP-CI73T) linked to interstitial lung disease promotes a late block in macroautophagy disrupting cellular proteostasis and mitophagy

Lysosomal storage diseases as disorders of autophagy

(2014) Carmine Settembre et al.   Autophagy

Does bafilomycin A1block the fusion of autophagosomes with lysosomes?

(2014) Daniel J. Klionsky et al.   Autophagy

Disruption of N-linked glycosylation promotes proteasomal degradation of the human ATP-binding cassette transporter ABCA3

(2013) Michael F. Beers et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Lung Fibrosis-associated Surfactant Protein A1 and C Variants Induce Latent Transforming Growth Factor β1 Secretion in Lung Epithelial Cells

(2013) Meenakshi Maitra et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The autophagosome: origins unknown, biogenesis complex

(2013) Christopher A. Lamb et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Mucopolysaccharidoses and Other Lysosomal Storage Diseases

(2013) Christina Lampe et al.   RHEUMATIC DISEASE CLINICS OF NORTH AMERICA

Impaired Autophagy in the Lipid-Storage Disorder Niemann-Pick Type C1 Disease

(2013) Sovan Sarkar et al.   Cell Reports

Insufficient autophagy in idiopathic pulmonary fibrosis

(2012) Jun Araya et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

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

(2012) Gareth A. Stewart et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Autophagy in lysosomal storage disorders

(2012) Andrew P. Lieberman et al.   Autophagy

Guidelines for the use and interpretation of assays for monitoring autophagy

(2012) Daniel J. Klionsky et al.   Autophagy

Autophagy in Idiopathic Pulmonary Fibrosis

(2012) Avignat S. Patel et al.   PLoS One

Analysis of Proteolytic Processes and Enzymatic Activities in the Generation of Huntingtin N-Terminal Fragments in an HEK293 Cell Model

(2012) Andrew T. N. Tebbenkamp et al.   PLoS One

High-resolution structure of a BRICHOS domain and its implications for anti-amyloid chaperone activity on lung surfactant protein C

(2012) H. Willander et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The ubiquitin E3 ligase parkin regulates the proapoptotic function of Bax

(2012) B. N. Johnson et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

(2011) Elena N. Atochina-Vasserman et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

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

(2011) Hanna Willander et al.   FEBS Journal

Multiple ways to die: Delineation of the unfolded protein response and apoptosis induced by Surfactant Protein C BRICHOS mutants

(2011) Jean Ann Maguire et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

A novel conserved targeting motif found in ABCA transporters mediates trafficking to early post-Golgi compartments

(2011) Michael F. Beers et al.   JOURNAL OF LIPID RESEARCH

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

Endoplasmic Reticulum Stress Induced by Surfactant Protein C BRICHOS Mutants Promotes Proinflammatory Signaling by Epithelial Cells

(2010) Jean Ann Maguire et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Impaired autophagosomes and lysosomes in neuronopathic Gaucher disease

(2010) Ying Sun et al.   Autophagy

PINK1/Parkin direct mitochondria to autophagy

(2010) Cristofol Vives-Bauza et al.   Autophagy

Uncovering the roles of PINK1 and Parkin in mitophagy

(2010) Noriyuki Matsuda et al.   Autophagy

Autophagosome maturation is impaired in Fabry disease

(2010) Marc Chévrier et al.   Autophagy

A non-BRICHOS surfactant protein c mutation disrupts epithelial cell function and intercellular signaling

(2010) Markus Woischnik et al.   BMC CELL BIOLOGY

Identification of Early Interstitial Lung Disease in an Individual With Genetic Variations in ABCA3 and SFTPC

(2010) Peter F. Crossno et al.   CHEST

Lysosomal fusion and SNARE function are impaired by cholesterol accumulation in lysosomal storage disorders

(2010) Alessandro Fraldi et al.   EMBO JOURNAL

Mitofusin 1 and mitofusin 2 are ubiquitinated in a PINK1/parkin-dependent manner upon induction of mitophagy

(2010) Matthew E. Gegg et al.   HUMAN MOLECULAR GENETICS

Regulation of Mammalian Autophagy in Physiology and Pathophysiology

(2010) Brinda Ravikumar et al.   PHYSIOLOGICAL REVIEWS

Aberrant Processing Forms of Lung Surfactant Proteins SP–B and SP–C Revealed by High-Resolution Mass Spectrometry

(2009) Dmitry Galetskiy et al.   EUROPEAN JOURNAL OF MASS SPECTROMETRY

Anterograde Transport of Surfactant Protein C Proprotein to Distal Processing Compartments Requires PPDY-mediated Association with Nedd4 Ubiquitin Ligases

(2009) Adam Kotorashvili et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Endoplasmic reticulum stress in alveolar epithelial cells is prominent in IPF: association with altered surfactant protein processing and herpesvirus infection

(2008) William E. Lawson et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Enhanced autophagy and mitochondrial aberrations in murine GM1-gangliosidosis

(2008) Ayumi Takamura et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

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

(2008) Charlotte Nerelius et al.   BIOCHEMICAL JOURNAL

Autophagy and multivesicular bodies: two closely related partners

(2008) C M Fader et al.   CELL DEATH AND DIFFERENTIATION

Trafficking and function of the tetraspanin CD63

(2008) Maaike S. Pols et al.   EXPERIMENTAL CELL RESEARCH

Parkin is recruited selectively to impaired mitochondria and promotes their autophagy

(2008) Derek Narendra et al.   JOURNAL OF CELL BIOLOGY

Population and Disease-Based Prevalence of the Common Mutations Associated With Surfactant Deficiency

(2008) Tami H Garmany et al.   PEDIATRIC RESEARCH

Adapting Proteostasis for Disease Intervention

(2008) William E. Balch et al.   SCIENCE