title: Increased cellular cholesterol efflux in glycogen storage disease type Ia mice: a potential mechanism that protects against premature atherosclerosis.
abstract: Glycogen storage disease type Ia (GSD-Ia) patients manifest a pro-atherogenic lipid profile but are not at elevated risk for developing atherosclerosis. Serum phospholipid, which correlates positively with the scavenger receptor class B type I (SR-BI)-mediated cholesterol efflux, and apolipoprotein A-IV and E, acceptors for ATP-binding cassette transporter A1 (ABCA1)-mediated cholesterol transport, are increased in GSD-Ia mice. Importantly, sera from GSD-Ia mice are more efficient than sera from control littermates in promoting SR-BI- and ABCA1-mediated cholesterol effluxes. As the first step in reverse cholesterol transport, essential for cholesterol homeostasis, these observations provide one explanation why GSD-Ia patients are apparently protected against premature atherosclerosis.
authors.affiliation: Section on Cellular Differentiation, Heritable Disorders Branch, National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 9D42, NIH 9000, Rockville Pike, Bethesda, MD 20892, USA.
authors.forename: Andrew D
authors.initials: AD
authors.lastname: Nguyen
authors.forename: Chi-Jiunn
authors.initials: CJ
authors.lastname: Pan
authors.forename: Jeng-Jer
authors.initials: JJ
authors.lastname: Shieh
authors.forename: Janice Yang
authors.initials: JY
authors.lastname: Chou
mesh_terms: D000818:Animals; D016632:Apolipoprotein A-I / Q000378:metabolism; D001054:Apolipoproteins A / Q000235:genetics / Q000378:metabolism; D001161:Arteriosclerosis / Q000378:metabolism* / Q000517:prevention & control; D002460:Cell Line; D002784:Cholesterol / Q000378:metabolism*; D005952:Glucose-6-Phosphatase / Q000235:genetics / Q000378:metabolism; D005953:Glycogen Storage Disease Type I / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D007527:Isoenzymes / Q000235:genetics / Q000378:metabolism; D008055:Lipids / Q000097:blood; D008264:Macrophages / Q000378:metabolism; D051379:Mice; D018345:Mice, Knockout
chemical_list: D016632:Apolipoprotein A-I; D001054:Apolipoproteins A; D007527:Isoenzymes; D008055:Lipids; C022205:apolipoprotein A-IV; D002784:Cholesterol; D005952:Glucose-6-Phosphatase