Misfolded BRICHOS SP-C mutant proteins induce apoptosis via caspase-4- and cytochrome c-related mechanisms. - Wikidata - awgldk - TriplyDB

Misfolded BRICHOS SP-C mutant proteins induce apoptosis via caspase-4- and cytochrome c-related mechanisms.

Misfolded BRICHOS SP-C mutant proteins induce apoptosis via caspase-4- and cytochrome c-related mechanisms.

http://www.wikidata.org/entity/Q40116328

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ERdj4 and ERdj5 are required for endoplasmic reticulum-associated protein degradation of misfolded surfactant protein C Meckel-Gruber syndrome protein MKS3 is required for endoplasmic reticulum-associated degradation of surfactant protein C Genetic interstitial lung disease Understanding Idiopathic Interstitial Pneumonia: A Gene-Based Review of Stressed Lungs Cellular mechanisms of tissue fibrosis. 7. New insights into the cellular mechanisms of pulmonary fibrosis Genetic disorders of surfactant dysfunction Alveolar surfactant homeostasis and the pathogenesis of pulmonary disease Chemical chaperones improve protein secretion and rescue mutant factor VIII in mice with hemophilia A Human surfactant protein A2 gene mutations impair dimmer/trimer assembly leading to deficiency in protein sialylation and secretion XBP-1-deficient plasmablasts show normal protein folding but altered glycosylation and lipid synthesis.A non-BRICHOS surfactant protein c mutation disrupts epithelial cell function and intercellular signaling Identification of early interstitial lung disease in an individual with genetic variations in ABCA3 and SFTPC.Surfactant protein A2 mutations associated with pulmonary fibrosis lead to protein instability and endoplasmic reticulum stress.Interstitial lung diseases in children.The surfactant protein C mutation A116D alters cellular processing, stress tolerance, surfactant lipid composition, and immune cell activation.Mutant surfactant A2 proteins associated with familial pulmonary fibrosis and lung cancer induce TGF-β1 secretion.Itm2a is a Pax3 target gene, expressed at sites of skeletal muscle formation in vivo.A non-BRICHOS SFTPC mutant (SP-CI73T) linked to interstitial lung disease promotes a late block in macroautophagy disrupting cellular proteostasis and mitophagy Genetics in pulmonary fibrosis--familial cases provide clues to the pathogenesis of idiopathic pulmonary fibrosis.The three R's of lung health and disease: repair, remodeling, and regeneration.Endoplasmic reticulum stress enhances fibrotic remodeling in the lungs A novel conserved targeting motif found in ABCA transporters mediates trafficking to early post-Golgi compartments.A nonaggregating surfactant protein C mutant is misdirected to early endosomes and disrupts phospholipid recycling.Role of endoplasmic reticulum stress in epithelial-mesenchymal transition of alveolar epithelial cells: effects of misfolded surfactant protein.Surfactant dysfunction.Genetic Basis of Children's Interstitial Lung Disease.Emerging evidence for endoplasmic reticulum stress in the pathogenesis of idiopathic pulmonary fibrosis.Lost after translation: insights from pulmonary surfactant for understanding the role of alveolar epithelial dysfunction and cellular quality control in fibrotic lung disease.Surfactant Protein C-associated interstitial lung disease; three different phenotypes of the same SFTPC mutation.Clinical and ultrastructural spectrum of diffuse lung disease associated with surfactant protein C mutations.Abrogation of ER stress-induced apoptosis of alveolar epithelial cells by angiotensin 1-7.Lung fibrosis-associated surfactant protein A1 and C variants induce latent transforming growth factor β1 secretion in lung epithelial cells.The expression of HoxB5 and SPC in neonatal rat lung after exposure to fluoxetine The Witschi Hypothesis revisited after 35 years: genetic proof from SP-C BRICHOS domain mutations Disruption of N-linked glycosylation promotes proteasomal degradation of the human ATP-binding cassette transporter ABCA3.Single-cell RNA sequencing identifies diverse roles of epithelial cells in idiopathic pulmonary fibrosis.Endoplasmic reticulum stress, a new wrestler, in the pathogenesis of idiopathic pulmonary fibrosis.The unfolded protein response in lung disease.BRICHOS domain associated with lung fibrosis, dementia and cancer--a chaperone that prevents amyloid fibril formation?Lung surfactant metabolism: early in life, early in disease and target in cell therapy.

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P2860

Misfolded BRICHOS SP-C mutant proteins induce apoptosis via caspase-4- and cytochrome c-related mechanisms.

http://www.wikidata.org/entity/Q40116328

description

2007 nî lūn-bûn

@nan

2007年の論文

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2007年学术文章

@wuu

2007年学术文章

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2007年学术文章

@zh-hans

2007年学术文章

@zh-my

2007年学术文章

@zh-sg

2007年學術文章

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2007年學術文章

@zh

2007年學術文章

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name

Misfolded BRICHOS SP-C mutant ...... tochrome c-related mechanisms.

@en

type

label

Misfolded BRICHOS SP-C mutant ...... tochrome c-related mechanisms.

@en

prefLabel

Misfolded BRICHOS SP-C mutant ...... tochrome c-related mechanisms.

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AJPLUNG.00025.2007

P1433

American Journal of Physiology - Lung Cellular and Molecular Physiology

P1476

Misfolded BRICHOS SP-C mutant ...... tochrome c-related mechanisms.

@en

P2093

Adam Kotorashvili

http://www.w3.org/2001/XMLSchema#string

Jean Ann Maguire

http://www.w3.org/2001/XMLSchema#string

Jennifer L Newitt

http://www.w3.org/2001/XMLSchema#string

Michael F Beers

http://www.w3.org/2001/XMLSchema#string

Scott J Russo

http://www.w3.org/2001/XMLSchema#string

Surafel Mulugeta

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P2860

Caspase-12 mediates endoplasmic-reticulum-specific apoptosis and cytotoxicity by amyloid-beta

Activation of caspase-12, an endoplastic reticulum (ER) resident caspase, through tumor necrosis factor receptor-associated factor 2-dependent mechanism in response to the ER stress

ASK1 is essential for endoplasmic reticulum stress-induced neuronal cell death triggered by expanded polyglutamine repeats

Cytochrome c and dATP-dependent formation of Apaf-1/caspase-9 complex initiates an apoptotic protease cascade

Involvement of caspase-4 in endoplasmic reticulum stress-induced apoptosis and Abeta-induced cell death

A surfactant protein C precursor protein BRICHOS domain mutation causes endoplasmic reticulum stress, proteasome dysfunction, and caspase 3 activation

A mutation in the surfactant protein C gene associated with familial interstitial lung disease

The Brichos domain-containing C-terminal part of pro-surfactant protein C binds to an unfolded poly-val transmembrane segment

Interstitial lung disease in a baby with a de novo mutation in the SFTPC gene

Aggresomes: a cellular response to misfolded proteins

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L720-9

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scholarly article

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10.1152/AJPLUNG.00025.2007

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3

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17586700

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apoptotic process