SFTPC mutations cause SP-C degradation and aggregate formation without increasing ER stress.

SFTPC mutations cause SP-C degradation and aggregate formation without increasing ER stress.

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http://www.wikidata.org/entity/Q45345359
Understanding Idiopathic Interstitial Pneumonia: A Gene-Based Review of Stressed LungsGenetic risk factors associated with respiratory distress syndromeDiseases of pulmonary surfactant homeostasis.Sequencing of idiopathic pulmonary fibrosis-related genes reveals independent single gene associationsLost after translation: insights from pulmonary surfactant for understanding the role of alveolar epithelial dysfunction and cellular quality control in fibrotic lung disease.MUC5B expression and location in surfactant protein C mutations in children.Genetic architecture of human fibrotic diseases: disease risk and disease progression.The Witschi Hypothesis revisited after 35 years: genetic proof from SP-C BRICHOS domain mutationsSafety and Efficacy of Allogeneic Lung Spheroid Cells in a Mismatched Rat Model of Pulmonary Fibrosis.A novel surfactant protein C L55F mutation associated with interstitial lung disease alters subcellular localization of proSP-C in A549 cells.Defective pro-SFTPC causes pulmonary surfactant metabolism dysfunction 2 (SMDP2) and respiratory distress syndrome (RDS)Protein Folding and the Challenges of Maintaining Endoplasmic Reticulum Proteostasis in Idiopathic Pulmonary Fibrosis.Deciphering the mechanism of Q145H SFTPC mutation unmasks a splicing defect and explains the severity of the phenotype.A novel surfactant protein C mutation resulting in aberrant protein processing and altered subcellular localization causes infantile interstitial lung disease.Defective pro-SFTPC does not translocate from ER membrane to multivesicle body
P2860

SFTPC mutations cause SP-C degradation and aggregate formation without increasing ER stress.

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http://www.wikidata.org/entity/Q45345359
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2013 nî lūn-bûn
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SFTPC mutations cause SP-C deg ...... without increasing ER stress.
@en
SFTPC mutations cause SP-C deg ...... without increasing ER stress.
@nl
type
label
SFTPC mutations cause SP-C deg ...... without increasing ER stress.
@en
SFTPC mutations cause SP-C deg ...... without increasing ER stress.
@nl
prefLabel
SFTPC mutations cause SP-C deg ...... without increasing ER stress.
@en
SFTPC mutations cause SP-C deg ...... without increasing ER stress.
@nl
P1433
P1476
P2093
P2860
P304
P31
P356
P433
P478
P577
P698
P356
P1433
P1476
SFTPC mutations cause SP-C deg ...... without increasing ER stress.
@en
P2093
P2860
P304
P31
P356
P433
P478
P577
P698