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. |