review article | Q7318358 |
scholarly article | Q13442814 |
P2093 | author name string | Surafel Mulugeta | |
Michael F Beers | |||
Shin-Ichi Nureki | |||
P2860 | cites work | Autophagy in idiopathic pulmonary fibrosis | Q21134133 |
BRICHOS - a superfamily of multidomain proteins with diverse functions | Q21202152 | ||
Coupling of stress in the ER to activation of JNK protein kinases by transmembrane protein kinase IRE1 | Q22011167 | ||
Determinants of progression in idiopathic pulmonary fibrosis | Q23911352 | ||
ABCA3 is a lamellar body membrane protein in human lung alveolar type II cells | Q24291924 | ||
ASK1 is essential for endoplasmic reticulum stress-induced neuronal cell death triggered by expanded polyglutamine repeats | Q24298956 | ||
The deacetylase HDAC6 regulates aggresome formation and cell viability in response to misfolded protein stress | Q24301895 | ||
p62/SQSTM1 binds directly to Atg8/LC3 to facilitate degradation of ubiquitinated protein aggregates by autophagy | Q24312147 | ||
ERdj4 and ERdj5 are required for endoplasmic reticulum-associated protein degradation of misfolded surfactant protein C | Q24318838 | ||
The cloning of a human ABC gene (ABC3) mapping to chromosome 16p13.3 | Q24319037 | ||
HDAC6 and microtubules are required for autophagic degradation of aggregated huntingtin | Q24337155 | ||
Involvement of caspase-4 in endoplasmic reticulum stress-induced apoptosis and Abeta-induced cell death | Q24338605 | ||
The pulmonary surfactant protein C (SP-C) precursor is a type II transmembrane protein | Q24529579 | ||
Endoplasmic reticulum stress: cell life and death decisions. | Q24536108 | ||
Altered stability of pulmonary surfactant in SP-C-deficient mice | Q24626562 | ||
A surfactant protein C precursor protein BRICHOS domain mutation causes endoplasmic reticulum stress, proteasome dysfunction, and caspase 3 activation | Q24645087 | ||
Misfolded proteins partition between two distinct quality control compartments | Q24651093 | ||
One step at a time: endoplasmic reticulum-associated degradation | Q24658302 | ||
An official American Thoracic Society clinical practice guideline: classification, evaluation, and management of childhood interstitial lung disease in infancy | Q26824718 | ||
Mechanisms of mitophagy | Q27310261 | ||
High-resolution structure of a BRICHOS domain and its implications for anti-amyloid chaperone activity on lung surfactant protein C. | Q27677103 | ||
Structure of 20S proteasome from yeast at 2.4 A resolution | Q27735081 | ||
Autophagy in the Pathogenesis of Disease | Q27860558 | ||
Essential role for autophagy in life span extension | Q28085050 | ||
Adapting proteostasis for disease intervention | Q28131818 | ||
Surfactant protein A (SP-A): the alveolus and beyond | Q28142201 | ||
Infants and Young Children with Children's Interstitial Lung Disease | Q35716043 | ||
Right place, right time: the evolving role of herpesvirus infection as a "second hit" in idiopathic pulmonary fibrosis | Q35850237 | ||
Emerging evidence for endoplasmic reticulum stress in the pathogenesis of idiopathic pulmonary fibrosis | Q35902352 | ||
Targeting the UPR transcription factor XBP1 protects against Huntington's disease through the regulation of FoxO1 and autophagy | Q35909430 | ||
Immunoregulatory functions of surfactant proteins | Q35998804 | ||
Surfactant protein C biosynthesis and its emerging role in conformational lung disease | Q36041099 | ||
A common MUC5B promoter polymorphism and pulmonary fibrosis. | Q36048530 | ||
Role of endoplasmic reticulum stress in age-related susceptibility to lung fibrosis | Q36049753 | ||
The environmental dependency of protein folding best explains prion and amyloid diseases | Q36054435 | ||
Adaptation and increased susceptibility to infection associated with constitutive expression of misfolded SP-C. | Q36117094 | ||
Randomised controlled trial comparing prednisolone alone with cyclophosphamide and low dose prednisolone in combination in cryptogenic fibrosing alveolitis | Q36199377 | ||
Clinical and pathologic features of familial interstitial pneumonia | Q36234581 | ||
Role of endoplasmic reticulum stress and c-Jun NH2-terminal kinase pathways in inflammation and origin of obesity and diabetes | Q36321560 | ||
4-Phenylbutyric acid treatment rescues trafficking and processing of a mutant surfactant protein-C. | Q36370170 | ||
ABC A-subfamily transporters: structure, function and disease. | Q36422412 | ||
The alveolar epithelium determines susceptibility to lung fibrosis in Hermansky-Pudlak syndrome | Q36488735 | ||
The roles of PINK1, parkin, and mitochondrial fidelity in Parkinson's disease | Q36611426 | ||
Type 2 alveolar cells are stem cells in adult lung. | Q36966911 | ||
Abrogation of ER stress-induced apoptosis of alveolar epithelial cells by angiotensin 1-7. | Q37054471 | ||
The mammalian endoplasmic reticulum-associated degradation system | Q37122324 | ||
Modification of surfactant protein D by reactive oxygen-nitrogen intermediates is accompanied by loss of aggregating activity, in vitro and in vivo | Q37159201 | ||
GRP78 expression inhibits insulin and ER stress-induced SREBP-1c activation and reduces hepatic steatosis in mice | Q37170811 | ||
Structure of pulmonary surfactant membranes and films: the role of proteins and lipid-protein interactions. | Q37178486 | ||
From endoplasmic-reticulum stress to the inflammatory response | Q37225354 | ||
Surfactant protein C-deficient mice are susceptible to respiratory syncytial virus infection | Q37264759 | ||
Anterograde transport of surfactant protein C proprotein to distal processing compartments requires PPDY-mediated association with Nedd4 ubiquitin ligases | Q37269148 | ||
Targeting proteins for destruction by the ubiquitin system: implications for human pathobiology | Q37286190 | ||
The Witschi Hypothesis revisited after 35 years: genetic proof from SP-C BRICHOS domain mutations | Q37440509 | ||
Disruption of N-linked glycosylation promotes proteasomal degradation of the human ATP-binding cassette transporter ABCA3. | Q37440530 | ||
Future directions in idiopathic pulmonary fibrosis research. An NHLBI workshop report | Q37698325 | ||
Activated alveolar epithelial cells initiate fibrosis through autocrine and paracrine secretion of connective tissue growth factor | Q37707606 | ||
The role of ubiquitin-proteasome system in ageing. | Q37842831 | ||
BRICHOS domain associated with lung fibrosis, dementia and cancer--a chaperone that prevents amyloid fibril formation? | Q37888775 | ||
Functional and morphological impact of ER stress on mitochondria | Q38102949 | ||
Idiopathic pulmonary fibrosis: prevailing and evolving hypotheses about its pathogenesis and implications for therapy | Q28200083 | ||
A mutation in the surfactant protein C gene associated with familial interstitial lung disease | Q28201394 | ||
Genetic disorders of surfactant dysfunction | Q28235256 | ||
The Brichos domain-containing C-terminal part of pro-surfactant protein C binds to an unfolded poly-val transmembrane segment | Q28241042 | ||
A block of autophagy in lysosomal storage disorders | Q28252016 | ||
ERAD: the long road to destruction | Q28264823 | ||
Protein aggregation and neurodegenerative disease | Q28273600 | ||
Interstitial lung disease in a baby with a de novo mutation in the SFTPC gene | Q28275609 | ||
Association between the MUC5B promoter polymorphism and survival in patients with idiopathic pulmonary fibrosis | Q28291143 | ||
Aggresomes: a cellular response to misfolded proteins | Q28292275 | ||
HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS | Q28306195 | ||
Biosynthesis of surfactant protein C (SP-C). Sorting of SP-C proprotein involves homomeric association via a signal anchor domain | Q28565893 | ||
Identification of LBM180, a lamellar body limiting membrane protein of alveolar type II cells, as the ABC transporter protein ABCA3 | Q28570821 | ||
Surfactant protein composition of lamellar bodies isolated from rat lung | Q28577423 | ||
Processing of surfactant protein C requires a type II transmembrane topology directed by juxtamembrane positively charged residues | Q28583232 | ||
Autophagy as a regulated pathway of cellular degradation | Q29547914 | ||
Impairment of the ubiquitin-proteasome system by protein aggregation | Q29614556 | ||
Proteotoxic stress and inducible chaperone networks in neurodegenerative disease and aging | Q29614783 | ||
Telomerase mutations in families with idiopathic pulmonary fibrosis | Q29615671 | ||
Autophagy and aging | Q29616002 | ||
Mitochondrial fission, fusion, and stress | Q29616536 | ||
Cell death and endoplasmic reticulum stress: disease relevance and therapeutic opportunities | Q29617768 | ||
Autophagy in human health and disease | Q29620121 | ||
Malfolded protein structure and proteostasis in lung diseases. | Q30353585 | ||
Genetic defects in surfactant protein A2 are associated with pulmonary fibrosis and lung cancer. | Q30374030 | ||
Epithelial endoplasmic reticulum stress and apoptosis in sporadic idiopathic pulmonary fibrosis | Q30484036 | ||
Biosynthesis of surfactant protein C: characterization of aggresome formation by EGFP chimeras containing propeptide mutants lacking conserved cysteine residues | Q31810547 | ||
S-nitrosylation of surfactant protein-D controls inflammatory function | Q33384274 | ||
Aberrant processing forms of lung surfactant proteins SP-B and SP-C revealed by high-resolution mass spectrometry | Q33399034 | ||
Sodium channels in alveolar epithelial cells: molecular characterization, biophysical properties, and physiological significance | Q33547262 | ||
Epstein-Barr virus replication within pulmonary epithelial cells in cryptogenic fibrosing alveolitis. | Q33605234 | ||
Nedd4-2-mediated ubiquitination facilitates processing of surfactant protein-C | Q33658812 | ||
Identification of early interstitial lung disease in an individual with genetic variations in ABCA3 and SFTPC. | Q33776818 | ||
Surfactant protein A2 mutations associated with pulmonary fibrosis lead to protein instability and endoplasmic reticulum stress. | Q33991198 | ||
Pathway to lamellar bodies for surfactant protein A. | Q33994628 | ||
Variable phenotype associated with SP-C gene mutations: fatal case with the I73T mutation | Q61449854 | ||
ABCA3 inactivation in mice causes respiratory failure, loss of pulmonary surfactant, and depletion of lung phosphatidylglycerol | Q62070315 | ||
Alteration of the Pulmonary Surfactant System in Full-Term Infants with Hereditary ABCA3 Deficiency | Q62661453 | ||
Amyloid fibril formation by pulmonary surfactant protein C | Q64049690 | ||
Pulmonary Fibrosis—A possible mechanism | Q66995286 | ||
Azathioprine Combined with Prednisone in the Treatment of Idiopathic Pulmonary Fibrosis: A Prospective Double-blind, Randomized, Placebo-controlled Clinical Trial | Q67924519 | ||
Nitration of surfactant protein A results in decreased ability to aggregate lipids | Q71464424 | ||
Morphometric characteristics of cells in the alveolar region of mammalian lungs | Q71817608 | ||
Precursor-product relationship between rabbit type II cell lamellar bodies and alveolar surface-active material. Surfactant turnover time | Q71858039 | ||
Elevation of antibodies to cytomegalovirus and other herpes viruses in pulmonary fibrosis | Q73734162 | ||
Apoptotic crosstalk between the endoplasmic reticulum and mitochondria controlled by Bcl-2 | Q73816419 | ||
Nitrated SP-A does not enhance adherence of Pneumocystis carinii to alveolar macrophages | Q77649754 | ||
Mutations in the surfactant protein C gene associated with interstitial lung disease | Q77763687 | ||
Progressive lung disease and surfactant dysfunction with a deletion in surfactant protein C gene | Q79346142 | ||
Mutation of SFTPC in infantile pulmonary alveolar proteinosis with or without fibrosing lung disease | Q79816730 | ||
Selective autophagy: ubiquitin-mediated recognition and beyond | Q84974064 | ||
BRICHOS: a conserved domain in proteins associated with dementia, respiratory distress and cancer | Q34138325 | ||
The surfactant protein C mutation A116D alters cellular processing, stress tolerance, surfactant lipid composition, and immune cell activation | Q34212504 | ||
Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis | Q34275871 | ||
Reserve autophagic capacity in alveolar epithelia provides a replicative niche for influenza A virus. | Q34305576 | ||
ABCA3 gene mutations in newborns with fatal surfactant deficiency | Q34308818 | ||
Acute inducible ablation of GRP78 reveals its role in hematopoietic stem cell survival, lymphogenesis and regulation of stress signaling. | Q34314109 | ||
Mutant surfactant A2 proteins associated with familial pulmonary fibrosis and lung cancer induce TGF-β1 secretion | Q34316498 | ||
ABCA3 mutations associated with pediatric interstitial lung disease | Q34455684 | ||
Genotype-phenotype correlations for infants and children with ABCA3 deficiency | Q34480215 | ||
Functional and trafficking defects in ATP binding cassette A3 mutants associated with respiratory distress syndrome | Q34484976 | ||
Characterization and classification of ATP-binding cassette transporter ABCA3 mutants in fatal surfactant deficiency. | Q34564474 | ||
Unexplained neonatal respiratory distress due to congenital surfactant deficiency | Q34630544 | ||
Heterozygosity for ABCA3 mutations modifies the severity of lung disease associated with a surfactant protein C gene (SFTPC) mutation | Q34642720 | ||
Endoplasmic reticulum stress-mediated apoptosis in pancreatic beta-cells | Q34724292 | ||
Cigarette smoking: a risk factor for idiopathic pulmonary fibrosis | Q34737461 | ||
A non-BRICHOS SFTPC mutant (SP-CI73T) linked to interstitial lung disease promotes a late block in macroautophagy disrupting cellular proteostasis and mitophagy | Q34801109 | ||
Mitophagy selectively degrades individual damaged mitochondria after photoirradiation | Q34806021 | ||
Orchestrating the unfolded protein response in health and disease | Q35001604 | ||
The three R's of lung health and disease: repair, remodeling, and regeneration | Q35015756 | ||
Herpesvirus DNA is consistently detected in lungs of patients with idiopathic pulmonary fibrosis | Q35027515 | ||
Hydrophobic surfactant proteins in lung function and disease | Q35034144 | ||
PINK1 deficiency impairs mitochondrial homeostasis and promotes lung fibrosis. | Q35056685 | ||
Telomerase mutations in smokers with severe emphysema. | Q35056698 | ||
Endoplasmic reticulum stress enhances fibrotic remodeling in the lungs | Q35081648 | ||
Increased and prolonged pulmonary fibrosis in surfactant protein C-deficient mice following intratracheal bleomycin | Q35085416 | ||
Integrating mechanisms of pulmonary fibrosis | Q35106452 | ||
A novel conserved targeting motif found in ABCA transporters mediates trafficking to early post-Golgi compartments | Q35114017 | ||
Extensive phenotyping of individuals at risk for familial interstitial pneumonia reveals clues to the pathogenesis of interstitial lung disease. | Q35153490 | ||
A nonaggregating surfactant protein C mutant is misdirected to early endosomes and disrupts phospholipid recycling. | Q35163057 | ||
Alveolar epithelial cells undergo epithelial-to-mesenchymal transition in response to endoplasmic reticulum stress | Q35183034 | ||
Early alveolar epithelial dysfunction promotes lung inflammation in a mouse model of Hermansky-Pudlak syndrome | Q35219750 | ||
Role of endoplasmic reticulum stress in epithelial-mesenchymal transition of alveolar epithelial cells: effects of misfolded surfactant protein | Q35219868 | ||
Genetic mutations in surfactant protein C are a rare cause of sporadic cases of IPF | Q35536294 | ||
Telomere dysfunction causes alveolar stem cell failure | Q35549158 | ||
Autophagy: a potential therapeutic target in lung diseases | Q38109605 | ||
The autophagosome: origins unknown, biogenesis complex | Q38160660 | ||
Regulation of the proteasome: evaluating the lung proteasome as a new therapeutic target | Q38179456 | ||
Impaired autophagy in the lipid-storage disorder Niemann-Pick type C1 disease | Q38315736 | ||
Acute cigarette smoke exposure impairs proteasome function in the lung | Q39282499 | ||
Crosstalk between the ubiquitin-proteasome system and autophagy in a human cellular model of Alzheimer's disease | Q39301707 | ||
Cigarette smoke induces endoplasmic reticulum stress response and proteasomal dysfunction in human alveolar epithelial cells | Q39305277 | ||
Surfactant protein C G100S mutation causes familial pulmonary fibrosis in Japanese kindred. | Q39492784 | ||
A proposed nomenclature for pulmonary surfactant-associated proteins | Q39548005 | ||
Some ABCA3 mutations elevate ER stress and initiate apoptosis of lung epithelial cells | Q39610877 | ||
The surfactant lipid transporter ABCA3 is N-terminally cleaved inside LAMP3-positive vesicles | Q39652287 | ||
Endoplasmic reticulum stress induced by surfactant protein C BRICHOS mutants promotes proinflammatory signaling by epithelial cells | Q39704568 | ||
Multiple ways to die: delineation of the unfolded protein response and apoptosis induced by Surfactant Protein C BRICHOS mutants | Q39925250 | ||
Aberrant catalytic cycle and impaired lipid transport into intracellular vesicles in ABCA3 mutants associated with nonfatal pediatric interstitial lung disease | Q39954325 | ||
Caspase-12 and endoplasmic reticulum stress mediate neurotoxicity of pathological prion protein. | Q39962425 | ||
Cigarette smoke induces an unfolded protein response in the human lung: a proteomic approach | Q40035546 | ||
Misfolded BRICHOS SP-C mutant proteins induce apoptosis via caspase-4- and cytochrome c-related mechanisms | Q40116328 | ||
Nonspecific interstitial pneumonia, alveolar proteinosis, and abnormal proprotein trafficking resulting from a spontaneous mutation in the surfactant protein C gene | Q40489429 | ||
Expression of a Human Surfactant Protein C Mutation Associated with Interstitial Lung Disease Disrupts Lung Development in Transgenic Mice | Q40629149 | ||
Post-translational processing of surfactant protein-C proprotein: targeting motifs in the NH(2)-terminal flanking domain are cleaved in late compartments | Q40821326 | ||
Caspase-8 is required for cell death induced by expanded polyglutamine repeats. | Q40961806 | ||
Acute exacerbation of idiopathic pulmonary fibrosis associated with air pollution exposure. | Q41382505 | ||
Susceptibility of Hermansky-Pudlak mice to bleomycin-induced type II cell apoptosis and fibrosis. | Q42065927 | ||
Heterozygosity for a surfactant protein C gene mutation associated with usual interstitial pneumonitis and cellular nonspecific interstitial pneumonitis in one kindred | Q42675595 | ||
Pulmonary surfactant-associated polypeptide C in a mixed organic solvent transforms from a monomeric alpha-helical state into insoluble beta-sheet aggregates | Q42846226 | ||
Deletion of exon 4 from human surfactant protein C results in aggresome formation and generation of a dominant negative | Q44285468 | ||
Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders. | Q44286601 | ||
Familial interstitial disease with I73T mutation: A mid- and long-term study | Q44728463 | ||
SFTPC mutations cause SP-C degradation and aggregate formation without increasing ER stress | Q45345359 | ||
New surfactant protein C gene mutations associated with diffuse lung disease | Q47797387 | ||
A placebo-controlled trial of interferon gamma-1b in patients with idiopathic pulmonary fibrosis | Q48033849 | ||
Secretion of surfactant protein C, an integral membrane protein, requires the N-terminal propeptide. | Q50336430 | ||
Revealing the pathogenic and aging-related mechanisms of the enigmatic idiopathic pulmonary fibrosis. an integral model. | Q50676736 | ||
Trafficking of newly synthesized surfactant protein A in isolated rat alveolar type II cells. | Q50755821 | ||
Pneumonitis and emphysema in sp-C gene targeted mice. | Q51691303 | ||
Interstitial pneumonia in Hermansky-Pudlak syndrome: significance of florid foamy swelling/degeneration (giant lamellar body degeneration) of type-2 pneumocytes. | Q52541285 | ||
ABCA3 is critical for lamellar body biogenesis in vivo. | Q52579496 | ||
Insufficient autophagy in idiopathic pulmonary fibrosis. | Q53138134 | ||
Carboxyl-terminal fragments of Alzheimer beta-amyloid precursor protein accumulate in restricted and unpredicted intracellular compartments in presenilin 1-deficient cells. | Q53237328 | ||
Endoplasmic reticulum stress in alveolar epithelial cells is prominent in IPF: association with altered surfactant protein processing and herpesvirus infection. | Q53486707 | ||
Surfactant protein C mutations are the basis of a significant portion of adult familial pulmonary fibrosis in a dutch cohort. | Q54418482 | ||
A common mutation in the surfactant protein C gene associated with lung disease. | Q54676468 | ||
Idiopathic pulmonary fibrosis | Q56764887 | ||
ABCA3 as a Lipid Transporter in Pulmonary Surfactant Biogenesis | Q57591393 | ||
XBP-1 deficiency in the nervous system reveals a homeostatic switch to activate autophagy | Q60613336 | ||
P433 | issue | 6 | |
P921 | main subject | quality control | Q827792 |
pulmonary surfactant | Q28163641 | ||
P304 | page(s) | L507-25 | |
P577 | publication date | 2015-07-17 | |
P1433 | published in | American Journal of Physiology - Lung Cellular and Molecular Physiology | Q4352992 |
P1476 | title | Lost after translation: insights from pulmonary surfactant for understanding the role of alveolar epithelial dysfunction and cellular quality control in fibrotic lung disease | |
P478 | volume | 309 |
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