Published ahead of print on April 21, 2005, doi:10.1165/rcmb.2004-0335OC Am. J. Respir. Cell Mol. Biol., Volume 33, Number 1, July 2005, 41-47 A more recent version of this article appeared on July 1, 2005
Submitted on October 28, 2004 Identification and Analysis of Axonemal Dynein Light Chain 1 in Primary Ciliary Dyskinesia PatientsJudit Horvath1,1 Department of Pediatrics and Adolescent Medicine, Albert-Ludwigs-University, Freiburg, Germany, 2 Institut fur Molekularbiologie, Medizinische Hochschule, Hannover, Germany, 3 Department of Biochemistry, University of Connecticut Health Center, Farmington CT, USA, 4 Department of Paediatrics and Child Health, Royal Free and University College Medical School, London, United Kingdom, 5 Department of Medicine, University of North Carolina, Chapel Hill, USA, 6 Max-Planck Institut for Molecular Genetics, Berlin, Germany, 7 Second Department of Pediatrics, School of Medicine, Semmelweis University, Budapest, Hungary, 8 Institute of Human Genetics, University of Goettingen, Goettingen, Germany * To whom correspondence should be addressed. E-mail: omran{at}kikli.ukl.uni-freiburg.de.
Primary ciliary dyskinesia (PCD) is a genetically heterogeneous disorder characterized by chronic infections of the upper and lower airways, randomization of left/right body asymmetry and reduced fertility. The phenotype results from dysfunction of motile cilia of the respiratory epithelium, at the embryonic node and of sperm flagella. Ultrastructural defects often involve outer dynein arms (ODAs), that are composed of several light (LCs), intermediate and heavy (HCs) dynein chains. We recently showed that recessive mutations of DNAH5, the human ortholog of the biflagellate Chlamydomonas ODA
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-HC, cause PCD. In Chlamydomonas motor protein activity of the 
