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Publication : Targeted disruption of NDST-1 gene leads to pulmonary hypoplasia and neonatal respiratory distress in mice.

First Author  Fan G Year  2000
Journal  FEBS Lett Volume  467
Issue  1 Pages  7-11
PubMed ID  10664446 Mgi Jnum  J:60280
Mgi Id  MGI:1353121 Doi  10.1016/s0014-5793(00)01111-x
Citation  Fan G, et al. (2000) Targeted disruption of NDST-1 gene leads to pulmonary hypoplasia and neonatal respiratory distress in mice. FEBS Lett 467(1):7-11
abstractText  In order to address the biological function of GlcNAc N-deacetylase/N-sulfotransferase-1 (NDST-1), we disrupted the NDST-1 gene by homologous recombination in mouse embryonic stem cells. The NDST-1 null mice developed respiratory distress and atelectasis that subsequently caused neonatal death. Morphological examination revealed type II pneumocyte immaturity, which was characterized by an increased glycogen content and a reduced number of lamellar bodies and microvilli. Biochemical analysis further indicated that both total phospholipids and disaturated phosphatidylcholine were reduced in the mutant lung. Our data revealed that NDST-1 was essential for the maturation of type II pneumocytes and its inactivation led to a neonatal respiratory distress syndrome.
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