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Targeted disruption of NDST‐1 gene leads to pulmonary hypoplasia and neonatal respiratory distress in mice
Author(s) -
Fan Guoping,
Xiao Lei,
Cheng Lu,
Wang Xinhui,
Sun Bo,
Hu Gengxi
Publication year - 2000
Publication title -
febs letters
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.593
H-Index - 257
eISSN - 1873-3468
pISSN - 0014-5793
DOI - 10.1016/s0014-5793(00)01111-x
Subject(s) - respiratory distress , hypoplasia , pulmonary hypoplasia , respiratory system , medicine , distress , biology , genetics , pediatrics , anesthesia , fetus , pregnancy , clinical psychology
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.