Use of knockout mice to study surfactant protein structure and function

Biology of the Neonate

Volumn 76, Issue SUPPL. 1, 1999, Pages 15-18

  Weaver, Timothy E ^a   Beck, David C ^a  

^a CINCINNATI CHILDREN'S HOSPITAL MEDICAL CENTER   (United States)

Author keywords

Gene replacement; Lung; Structure function, surfactant protein; Transgenic mice; Type II cell

Indexed keywords

LUNG SURFACTANT; SURFACTANT PROTEIN B;

CONFERENCE PAPER; GENE MUTATION; HOMEOSTASIS; KNOCKOUT MOUSE; MOUSE; NONHUMAN; PATHOPHYSIOLOGY; PRIORITY JOURNAL; PROTEIN STRUCTURE; STRUCTURE ACTIVITY RELATION;

ANIMALS; DIMERIZATION; HUMANS; LUNG; MICE; MICE, KNOCKOUT; MICE, TRANSGENIC; MUTAGENESIS; PROTEOLIPIDS; PULMONARY SURFACTANTS;

MUS MUSCULUS;

EID: 0032973035     PISSN: 00063126     EISSN: None     Source Type: Journal    
DOI: 10.1159/000047041     Document Type: Conference Paper

References (10)

1. Whitsett JA, Nogee LM, Weaver TE, Horowitz AD: Human surfactant protein B: Structure, function, regulation, and genetic disease. Physiol Rev 1995;75:749-757.
2. Weaver TE, Whitsett JA: Function and regulation of expression of pulmonary surfactant-associated proteins. Biochem J 1991;273:249-264.
3. Nogee LM, DeMello DE, Dehner LP, Colten HR: Deficiency of pulmonary surfactant protein B in congenital alveolar proteinosis. N Engl J Med 1993;328:406-410.
4. Nogee LM, Garnier G, Dietz HC, Singer L, Murphy AM, Demello DE, Colten HR: A mutation in the surfactant protein B gene responsible for fatal neonatal respiratory disease in multiple kindreds. J Clin Invest 1994;93:1860-1863.
5. Lin S, Akinbi HT, Breslin JS, Weaver TE: Structural requirements for targeting of surfactant protein B (SP-B) to secretory granules in vitro and in vivo. J Biol Chem 1996;271: 19689-19695.
6. Lin S, Phillips KS, Wilder MR, Weaver TE: Structural requirements for intracellular transport of pulmonary surfactant protein B (SP-B). Biochim Biophys Acta 1996;1312: 177-185.
7. Akinbi HT, Breslin JS, Ikegami M, Iwamoto HS, Clark JC, Whitsett JA, Jobe AH, Weaver TE: Rescue of SP-B knockout mice with a truncated SP-B proprotein - function of the C-terminal propeptide. J Biol Chem 1997;272:9640-9647.
8. Glasser SW, Korfhagen TR, Wert SE, Bruno MD, McWilliams KM, Vorbroker DK, Whitsett JA: Genetic element from human surfactant protein SP-C gene confers bronchiolar-alveolar cell specificity in transgenic mice. Am J Physiol 1991;261: L349-L356.
9. Clark JC, Wert SE, Bachurski CJ, Stahlman MT, Stripp BR, Weaver TE, Whitsett JA: Targeted disruption of the surfactant protein B gene disrupts surfactant homeostasis. causing respiratory failure in newborn mice. Proc Natl Acad Sci USA 1995;92:7794-7798.
10. Hanks M, Wurst W, Anson-Cartwright L, Auerbach AB, Joyner AL: Rescue of the En-1 mutant phenotype by replacement of En-1 with En-2. Science 1995;269:679-682.