Very low surfactant protein C contents in newborn Belgian White and Blue calves with respiratory distress syndrome

Danlois, Fabien; Zaltash, Shahparak; Johansson, Jan; Robertson, Bengt; Haagsman, Henk P.; Eijk, Martin van; Beers, Michael F.; Ruysschaert, J. M.; Vandenbussche, Guy

doi:10.1042/0264-6021:3510779

Cited by 23 publications

(4 citation statements)

References 44 publications

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“…SP-C deficits are related to acute and chronic infant lung diseases in humans 27 and respiratory failure in cattle. 28 Furthermore, mutations in proSP-C have been correlated with chronic interstitial pneumonia. 11,12 ProSP-C is an type II integral membrane protein with structural homology to the amyloidogenic BRI family of proteins, which cause neurodegenerative dementia.…”

Section: Discussionmentioning

confidence: 99%

Aberrant quality control in the endoplasmic reticulum impairs the biosynthesis of pulmonary surfactant in mice expressing mutant BiP

et al. 2007

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Accumulation of misfolded proteins in the endoplasmic reticulum (ER) induces the unfolded protein response (UPR), which alleviates protein overload in the secretory pathway. Although the UPR is activated under diverse pathological conditions, its physiological role during development and in adulthood has not been fully elucidated. Binding immunoglobulin protein (BiP) is an ER chaperone, which is central to ER function. We produced knock-in mice expressing a mutant BiP lacking the retrieval sequence to cause a defect in ER function without completely eliminating BiP. In embryonic fibroblasts, the UPR compensated for mutation of BiP. However, neonates expressing mutant BiP suffered respiratory failure due to impaired secretion of pulmonary surfactant by alveolar type II epithelial cells. Expression of surfactant protein (SP)-C was reduced and the lamellar body was malformed, indicating that BiP plays a critical role in the biosynthesis of pulmonary surfactant. Because pulmonary surfactant requires extensive post-translational processing in the secretory pathway, these findings suggest that in secretory cells, such as alveolar type II cells, the UPR is essential for managing the normal physiological ER protein overload that occurs during development. Moreover, failure of this adaptive mechanism may increase pulmonary susceptibility to environmental insults, such as hypoxia and ischemia, ultimately leading to neonatal respiratory failure.

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Section: Discussionmentioning

confidence: 99%

Aberrant quality control in the endoplasmic reticulum impairs the biosynthesis of pulmonary surfactant in mice expressing mutant BiP

et al. 2007

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“…Therefore, an intra-alveolar accumulation of precursors of SP-B occurs under various pathological conditions and may not be due to a specific protein-protein interaction of mutant proSP-C and wild-type proSP-B. Genetically engineered mice, as well as newborn calves of the Belgian White and Blue breed, with a lack or deficiency of SP-C, develop interstitial lung disease during postnatal growth [13,42,43]. However, anti-SP-C readily identified mature mono-and dimeric SP-C forms in BAL fluids from the index patient, providing indirect evidence that the heterozygous g.1286TwC (I73T) missense mutation did not have a dominant-negative effect on SP-C biosynthesis.…”

Section: Discussionmentioning

confidence: 99%

Interstitial lung disease in a baby with ade novomutation in the SFTPC gene

Brasch

Griese²,

Tredano³

et al. 2004

Eur Respir J

144

132

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Mutations in the surfactant protein C gene (SFTPC) were recently reported in patients with interstitial lung disease.In a 13‐month-old infant with severe respiratory insufficiency, a lung biopsy elicited combined histological patterns of nonspecific interstitial pneumonia and pulmonary alveolar proteinosis. Immunohistochemical and biochemical analyses showed an intra-alveolar accumulation of surfactant protein (SP)‐A, precursors of SP‐B, mature SP‐B, aberrantly processed proSP‐C, as well as mono- and dimeric SP‐C.Sequencing of genomic DNA detected ade novoheterozygous missense mutation of the SFTPC gene (g.1286T>C) resulting in a substitution of threonine for isoleucine (I73T) in the C‐terminal propeptide. At the ultrastructural level, abnormal transport vesicles were detected in type‐II pneumocytes. Fusion proteins, consisting of enhanced green fluorescent protein and wild-type or mutant proSP‐C, were used to evaluate protein traffickingin vitro. In contrast to wild-type proSP‐C, mutant proSP‐C was routed to early endosomes when transfected into A549 epithelial cells.In contrast to previously reported mutations, the I73T represents a new class of surfactant protein C gene mutations, which is marked by a distinct trafficking, processing, palmitoylation, and secretion of the mutant and wild-type surfactant protein C. This report heralds the emerging diversity of phenotypes associated with the expression of mutant surfactant C proteins.

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“…Targeted disruption of SP-B causes respiratory failure in newborn mice (4). A lack of SP-C is associated with respiratory distress syndrome (5,6). Regulation of SP gene expression is multifactorial, involving glucocorticoid hormones (8), retinoic acid (9), fibroblast growth factor (FGF)-2 (10), transforming growth factor-␤ (11), nitric oxide, and keratinocyte growth factor (12).…”

mentioning

confidence: 99%

Hypoxia-Induced Mitogenic Factor Modulates Surfactant Protein B and C Expression in Mouse Lung

Tong

Zheng

Dodd‐o

et al. 2006

Am J Respir Cell Mol Biol

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Very low surfactant protein C contents in newborn Belgian White and Blue calves with respiratory distress syndrome

Cited by 23 publications

References 44 publications

Aberrant quality control in the endoplasmic reticulum impairs the biosynthesis of pulmonary surfactant in mice expressing mutant BiP

Aberrant quality control in the endoplasmic reticulum impairs the biosynthesis of pulmonary surfactant in mice expressing mutant BiP

Interstitial lung disease in a baby with ade novomutation in the SFTPC gene

Hypoxia-Induced Mitogenic Factor Modulates Surfactant Protein B and C Expression in Mouse Lung

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