Targeted disruption of NDST‐1 gene leads to pulmonary hypoplasia and neonatal respiratory distress in mice

FEBS Letters

Sun, Bo¹  Fan, Guoping⁴  Wang, Xinhui²  Cheng, Lu³  Hu, Gengxi³  Xiao, Lei³ 

[1] Children's Hospital Research Institute, Shanghai Medical University, Shanghai 200032, PR China;School of Life Sciences, University of Science and Technology of China, Hefei 230027, PR China;Max-Planck Guest Laboratory, Shanghai Institute of Cell Biology, Chinese Academy of Science, 320 Yueyang Road, Shanghai 200031, PR China;Whitehead Institute for Biomedical Research, Cambridge, MA 02142, USA

关键词: NDST-1;    Disruption;    Atelectasis;    Type II pneumocyte;    Immaturity;    Respiratory distress syndrome;   

DOI  :  10.1016/S0014-5793(00)01111-X

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

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.

【 授权许可】

Unknown