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

American Journal of Physiology - Lung Cellular and Molecular Physiology

Volumn 295, Issue 4, 2008, Pages

  Matsumura, Yoshihiro ^a,d   Ban, Nobuhiro ^a,b,c   Inagaki, Nobuya ^a,b,c  

^a AKITA UNIVERSITY SCHOOL OF MEDICINE   (Japan)

^b KYOTO UNIVERSITY   (Japan)

^c JAPAN SCIENCE AND TECHNOLOGY AGENCY   (Japan)

^d OREGON HEALTH AND SCIENCE UNIVERSITY   (United States)

Author keywords

ATP binding cassette A3 mutant; Lamellar body; Lipid transporter; Pediatric interstitial lung disease; Phosphatidylcholine

Indexed keywords

ABC TRANSPORTER A1; ABC TRANSPORTER A3; ADENOSINE TRIPHOSPHATE; CHOLINE; LUNG SURFACTANT; MUTANT PROTEIN; NUCLEOTIDE BINDING PROTEIN; PHOSPHOLIPID; UNCLASSIFIED DRUG; VANADIC ACID; ABC TRANSPORTER; ABCA3 PROTEIN, HUMAN; LIPID; RECOMBINANT PROTEIN;

AMINO ACID SEQUENCE; ARTICLE; CATALYSIS; CELL VACUOLE; CELLULAR DISTRIBUTION; CONTROLLED STUDY; DISEASE ASSOCIATION; DISEASE SEVERITY; EMBRYO; FRACTIONATION; GENE MUTATION; GLYCOSYLATION; HUMAN; HUMAN CELL; HYDROLYSIS; INTERSTITIAL LUNG DISEASE; LAMELLAR BODY; LIPID ANALYSIS; LIPID TRANSPORT; LUNG ALVEOLUS CELL TYPE 2; NEWBORN; PATHOPHYSIOLOGY; PHENOTYPE; PHOTOAFFINITY LABELING; PRIORITY JOURNAL; PROTEIN ANALYSIS; PROTEIN FUNCTION; PROTEIN LOCALIZATION; PROTEIN MOTIF; PROTEIN TRANSPORT; SEQUENCE HOMOLOGY; SUCROSE GRADIENT; WILD TYPE; CELL LINE; CHEMISTRY; CHILD; GENETIC TRANSFECTION; GENETICS; KIDNEY; METABOLISM; PHYSIOLOGY; PLASMID; PRENATAL DEVELOPMENT; REPORTER GENE; TRANSPORT AT THE CELLULAR LEVEL;

ATP-BINDING CASSETTE TRANSPORTERS; BIOLOGICAL TRANSPORT; CATALYSIS; CELL LINE; CHILD; GENES, REPORTER; GLYCOSYLATION; HUMANS; KIDNEY; LIPIDS; LUNG DISEASES, INTERSTITIAL; PLASMIDS; RECOMBINANT PROTEINS; TRANSFECTION; VANADATES;

EID: 56149098789     PISSN: 10400605     EISSN: 15221504     Source Type: Journal    
DOI: 10.1152/ajplung.90352.2008     Document Type: Article

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