Selective reconstitution of liver cholesterol biosynthesis promotes lung maturation but does not prevent neonatal lethality in Dhcr7 null mice

[1]  P. Maček,et al.  Erratum to “Steroid structural requirements for interaction of ostreolysin, a lipid-raft binding cytolysin, with lipid monolayers and bilayers” [Biochim. Biophys. Acta 1758 (2006) 1662–1670] , 2007 .

[2]  I. Kochevar,et al.  7-Dehydrocholesterol enhances ultraviolet A-induced oxidative stress in keratinocytes: roles of NADPH oxidase, mitochondria, and lipid rafts. , 2006, Free radical biology & medicine.

[3]  P. Maček,et al.  Steroid structural requirements for interaction of ostreolysin, a lipid-raft binding cytolysin, with lipid monolayers and bilayers. , 2006, Biochimica et biophysica acta.

[4]  Megha,et al.  Cholesterol Precursors Stabilize Ordinary and Ceramide-rich Ordered Lipid Domains (Lipid Rafts) to Different Degrees , 2006, Journal of Biological Chemistry.

[5]  E. Babiychuk,et al.  Biochemical characterization of detergent-resistant membranes: a systematic approach. , 2006, The Biochemical journal.

[6]  Hongwei Yu,et al.  The use of the Dhcr7 knockout mouse to accurately determine the origin of fetal sterols Published, JLR Papers in Press, May 1, 2006. , 2006, Journal of Lipid Research.

[7]  J. Rivera,et al.  Cholesterol deficiency in a mouse model of Smith-Lemli-Opitz syndrome reveals increased mast cell responsiveness , 2006, The Journal of experimental medicine.

[8]  R. Steiner,et al.  A membrane defect in the pathogenesis of the Smith-Lemli-Opitz syndrome Published, JLR Papers in Press, October 28, 2005. , 2006, Journal of Lipid Research.

[9]  H. Yu,et al.  Recent insights into the Smith–Lemli–Opitz syndrome , 2005, Clinical genetics.

[10]  K. Gaus,et al.  Domain-specific lipid distribution in macrophage plasma membranes Published, JLR Papers in Press, May 1, 2005. DOI 10.1194/jlr.M500103-JLR200 , 2005, Journal of Lipid Research.

[11]  S. Fliesler,et al.  A comparison of the behavior of cholesterol and selected derivatives in mixed sterol-phospholipid Langmuir monolayers: a fluorescence microscopy study. , 2005, Chemistry and physics of lipids.

[12]  Hongwei Yu,et al.  Partial rescue of neonatal lethality of Dhcr7 null mice by a nestin promoter-driven DHCR7 transgene expression. , 2005, Brain research. Developmental brain research.

[13]  Kai Simons,et al.  Model systems, lipid rafts, and cell membranes. , 2004, Annual review of biophysics and biomolecular structure.

[14]  P. Morgan,et al.  A stomatin and a degenerin interact in lipid rafts of the nervous system of Caenorhabditis elegans. , 2004, American journal of physiology. Cell physiology.

[15]  Hongwei Yu,et al.  Late gestational lung hypoplasia in a mouse model of the Smith-Lemli-Opitz syndrome , 2004, BMC Developmental Biology.

[16]  S. Fliesler,et al.  Formation of 7-dehydrocholesterol-containing membrane rafts in vitro and in vivo, with relevance to the Smith-Lemli-Opitz syndrome Published, JLR Papers in Press, November 1, 2003. DOI 10.1194/jlr.M300232-JLR200 , 2004, Journal of Lipid Research.

[17]  F. Porter Human malformation syndromes due to inborn errors of cholesterol synthesis. , 2003, Current opinion in pediatrics.

[18]  Jonathan A. Cooper,et al.  Lipid-dependent Recruitment of Neuronal Src to Lipid Rafts in the Brain* , 2003, Journal of Biological Chemistry.

[19]  D. Russell,et al.  Knockout of the Cholesterol 24-Hydroxylase Gene in Mice Reveals a Brain-specific Mechanism of Cholesterol Turnover* , 2003, Journal of Biological Chemistry.

[20]  G. Herman Disorders of cholesterol biosynthesis: prototypic metabolic malformation syndromes. , 2003, Human molecular genetics.

[21]  H. Andersson,et al.  Desmosterolosis presenting with multiple congenital anomalies and profound developmental delay. , 2002, American journal of medical genetics.

[22]  Kai Simons,et al.  Cholesterol, lipid rafts, and disease. , 2002, The Journal of clinical investigation.

[23]  J. Opitz,et al.  Cholesterol and development: the RSH ("Smith-Lemli-Opitz") syndrome and related conditions. , 2002, Pediatric pathology & molecular medicine.

[24]  Hans R. Waterham,et al.  Mutations in the 3β-Hydroxysterol Δ24-Reductase Gene Cause Desmosterolosis, an Autosomal Recessive Disorder of Cholesterol Biosynthesis , 2001 .

[25]  N. Maeda,et al.  7-Dehydrocholesterol-dependent proteolysis of HMG-CoA reductase suppresses sterol biosynthesis in a mouse model of Smith-Lemli-Opitz/RSH syndrome. , 2001, The Journal of clinical investigation.

[26]  C. Vilchèze,et al.  Effect of the Structure of Natural Sterols and Sphingolipids on the Formation of Ordered Sphingolipid/Sterol Domains (Rafts) , 2001, The Journal of Biological Chemistry.

[27]  F. F. Weight,et al.  Biochemical, phenotypic and neurophysiological characterization of a genetic mouse model of RSH/Smith--Lemli--Opitz syndrome. , 2001, Human molecular genetics.

[28]  J. Slotte,et al.  Sterol-induced upregulation of phosphatidylcholine synthesis in cultured fibroblasts is affected by the double-bond position in the sterol tetracyclic ring structure. , 2000, European journal of biochemistry.

[29]  R. Hennekam,et al.  The Smith-Lemli-Opitz syndrome , 2000, Journal of medical genetics.

[30]  P. Vreken,et al.  Smith-Lemli-Opitz syndrome is caused by mutations in the 7-dehydrocholesterol reductase gene. , 1998, American journal of human genetics.

[31]  G. Utermann,et al.  Mutations in the Delta7-sterol reductase gene in patients with the Smith-Lemli-Opitz syndrome. , 1998, Proceedings of the National Academy of Sciences of the United States of America.

[32]  D. S. Lin,et al.  Mutations in the human sterol delta7-reductase gene at 11q12-13 cause Smith-Lemli-Opitz syndrome. , 1998, American journal of human genetics.

[33]  P. Morell,et al.  Sources of cholesterol during development of the rat fetus and fetal organs. , 1997, Journal of lipid research.

[34]  C. Rosenfeld,et al.  Brain does not utilize low density lipoprotein-cholesterol during fetal and neonatal development in the sheep. , 1996, Journal of lipid research.

[35]  G Salen,et al.  Defective cholesterol biosynthesis associated with the Smith-Lemli-Opitz syndrome. , 1994, The New England journal of medicine.

[36]  P. Veldhoven,et al.  Inorganic and organic phosphate measurements in the nanomolar range. , 1987, Analytical biochemistry.

[37]  L. Bagatolli,et al.  Cholesterol Rules DIRECT OBSERVATION OF THE COEXISTENCE OF TWO FLUID PHASES IN NATIVE PULMONARY SURFACTANT MEMBRANES AT PHYSIOLOGICAL TEMPERATURES* , 2004 .

[38]  P. Vreken,et al.  Mutations in the 3beta-hydroxysterol Delta24-reductase gene cause desmosterolosis, an autosomal recessive disorder of cholesterol biosynthesis. , 2001, American journal of human genetics.

[39]  S. Innis,et al.  Cholesterol synthesis and accretion within various tissues of the fetal and neonatal rat. , 2001, Metabolism: clinical and experimental.

[40]  F. Fahrenholz,et al.  Cholesterol binds to synaptophysin and is required for biogenesis of synaptic vesicles , 1999, Nature Cell Biology.