Pediatric Interstitial Lung Disease

2002 ◽  
Vol 165 (11) ◽  
pp. 1466-1467 ◽  
Author(s):  
Leland L. Fan ◽  
Claire Langston
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Pediatric Interstitial Lung Disease

Aberrant catalytic cycle and impaired lipid transport into intracellular vesicles in ABCA3 mutants associated with nonfatal pediatric interstitial lung disease

2008 ◽  
Vol 295 (4) ◽  
pp. L698-L707 ◽  
Author(s):  
Yoshihiro Matsumura ◽  
Nobuhiro Ban ◽  
Nobuya Inagaki
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Catalytic Cycle ◽  
Lipid Transport ◽  
Alveolar Type ◽  
Pathophysiological Mechanism ◽  
Wild Type ◽  
Mutant Proteins ◽  
Intracellular Vesicles ◽  
Pediatric Interstitial Lung Disease

The ATP-binding cassette transporter ABCA3 mediates uptake of choline-phospholipids into intracellular vesicles and is essential for surfactant metabolism in lung alveolar type II cells. We have shown previously that ABCA3 mutations in fatal surfactant deficiency impair intracellular localization or ATP hydrolysis of ABCA3 protein. However, the mechanisms underlying the less severe phenotype of patients with ABCA3 mutation are unclear. In this study, we characterized ABCA3 mutant proteins identified in pediatric interstitial lung disease (pILD). E292V (intracellular loop 1), E690K (adjacent to Walker B motif in nucleotide binding domain 1), and T1114M (8th putative transmembrane segment) mutant proteins are localized mainly in intracellular vesicle membranes as wild-type protein. Lipid analysis and sucrose gradient fractionation revealed that the transport function of E292V mutant protein is moderately preserved, whereas those of E690K and T1114M mutant proteins are severely impaired. Vanadate-induced nucleotide trapping and photoaffinity labeling of wild-type and mutant proteins using 8-azido-[32P]ATP revealed an aberrant catalytic cycle in these mutant proteins. These results demonstrate the importance of a functional catalytic cycle in lipid transport of ABCA3 and suggest a pathophysiological mechanism of pILD due to ABCA3 mutation.

scholarly journals Surfactant proteins in pediatric interstitial lung disease

2015 ◽  
Vol 79 (1) ◽  
pp. 34-41 ◽  
Author(s):  
Matthias Griese ◽  
Elke Lorenz ◽  
Meike Hengst ◽  
Andrea Schams ◽  
Traudl Wesselak ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Surfactant Proteins ◽  
Pediatric Interstitial Lung Disease

scholarly journals ABCA3Mutations Associated with Pediatric Interstitial Lung Disease

2005 ◽  
Vol 172 (8) ◽  
pp. 1026-1031 ◽  
Author(s):  
Janine E. Bullard ◽  
Susan E. Wert ◽  
Jeffrey A. Whitsett ◽  
Michael Dean ◽  
Lawrence M. Nogee
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Pediatric Interstitial Lung Disease

Pediatric Interstitial Lung Disease

CHEST Journal ◽  
2013 ◽  
Vol 144 (3) ◽  
pp. 728-730 ◽  
Author(s):  
Lael M. Yonker ◽  
T. Bernard Kinane
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Pediatric Interstitial Lung Disease

Serum KL-6 and Surfactant Proteins A and D in Pediatric Interstitial Lung Disease

CHEST Journal ◽  
2005 ◽  
Vol 127 (1) ◽  
pp. 403-407 ◽  
Author(s):  
Qasem A. Al-Salmi ◽  
Joseph N. Walter ◽  
Giuseppe N. Colasurdo ◽  
Marianna M. Sockrider ◽  
E. O'Brian Smith ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Surfactant Proteins ◽  
Pediatric Interstitial Lung Disease

Transbronchial Lung Cryobiopsy for Diagnosis of Pediatric Interstitial Lung Disease

2018 ◽  
Vol 55 (6) ◽  
pp. 519-520 ◽  
Author(s):  
J. T. Srikanta ◽  
S. Swarna ◽  
D. S. Shylendra ◽  
Ravindra Mehta
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Transbronchial Lung Cryobiopsy ◽  
Pediatric Interstitial Lung Disease

Pediatric interstitial lung disease revisited

2004 ◽  
Vol 38 (5) ◽  
pp. 369-378 ◽  
Author(s):  
Leland L. Fan ◽  
Robin R. Deterding ◽  
Claire Langston
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Pediatric Interstitial Lung Disease
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