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Increased lipogenesis and resistance of lipoproteins to oxidative modification in two patients with glycogen storage disease type 1a
We describe 2 patients with glycogen storage disease type 1a and severe hyperlipidemia without premature atherosclerosis. Susceptibility of low-density lipoproteins to oxidation was decreased, possibly related to the ~40-fold increase in palmitate synthesis altering lipoprotein saturated fatty acid...
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| Published in: | The Journal of pediatrics 2002-02, Vol.140 (2), p.256-260 |
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| Main Authors: | , , , , , , , , , |
| Format: | Article |
| Language: | English |
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| cited_by | cdi_FETCH-LOGICAL-c373t-c2c27d45a18caed972336f627ecdb4530d893d9626e06f137f478ea0493be0463 |
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| cites | cdi_FETCH-LOGICAL-c373t-c2c27d45a18caed972336f627ecdb4530d893d9626e06f137f478ea0493be0463 |
| container_end_page | 260 |
| container_issue | 2 |
| container_start_page | 256 |
| container_title | The Journal of pediatrics |
| container_volume | 140 |
| creator | Bandsma, Robert H.J. Rake, Jan-Peter Visser, Gepke Neese, Richard A. Hellerstein, Marc K. van Duyvenvoorde, Wim Princen, Hans M.G. Stellaard, Frans Smit, G.Peter A. Kuipers, Folkert |
| description | We describe 2 patients with glycogen storage disease type 1a and severe hyperlipidemia without premature atherosclerosis. Susceptibility of low-density lipoproteins to oxidation was decreased, possibly related to the ~40-fold increase in palmitate synthesis altering lipoprotein saturated fatty acid contents. These findings are potentially relevant for antihyperlipidemic treatment in patients with glycogen storage disease type 1a. (J Pediatr 2002;140:256-60) |
| doi_str_mv | 10.1067/mpd.2002.121382 |
| format | article |
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Susceptibility of low-density lipoproteins to oxidation was decreased, possibly related to the ~40-fold increase in palmitate synthesis altering lipoprotein saturated fatty acid contents. These findings are potentially relevant for antihyperlipidemic treatment in patients with glycogen storage disease type 1a. (J Pediatr 2002;140:256-60)</description><identifier>ISSN: 0022-3476</identifier><identifier>EISSN: 1097-6833</identifier><identifier>DOI: 10.1067/mpd.2002.121382</identifier><identifier>PMID: 11865283</identifier><identifier>CODEN: JOPDAB</identifier><language>eng</language><publisher>New York, NY: Elsevier Inc</publisher><subject>Adult ; Biological and medical sciences ; Cholesterol, LDL - metabolism ; Disorders of blood lipids. Hyperlipoproteinemia ; Glycogen Storage Disease Type I - blood ; Glycogen Storage Disease Type I - complications ; Glycogen Storage Disease Type I - genetics ; Glycogen Storage Disease Type I - physiopathology ; Humans ; Hyperlipidemias - complications ; Lipoproteins, VLDL - metabolism ; Male ; Medical sciences ; Metabolic diseases ; Oxidation-Reduction ; Triglycerides - blood</subject><ispartof>The Journal of pediatrics, 2002-02, Vol.140 (2), p.256-260</ispartof><rights>2002 Mosby, Inc.</rights><rights>2002 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c373t-c2c27d45a18caed972336f627ecdb4530d893d9626e06f137f478ea0493be0463</citedby><cites>FETCH-LOGICAL-c373t-c2c27d45a18caed972336f627ecdb4530d893d9626e06f137f478ea0493be0463</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27922,27923</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=13538147$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/11865283$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Bandsma, Robert H.J.</creatorcontrib><creatorcontrib>Rake, Jan-Peter</creatorcontrib><creatorcontrib>Visser, Gepke</creatorcontrib><creatorcontrib>Neese, Richard A.</creatorcontrib><creatorcontrib>Hellerstein, Marc K.</creatorcontrib><creatorcontrib>van Duyvenvoorde, Wim</creatorcontrib><creatorcontrib>Princen, Hans M.G.</creatorcontrib><creatorcontrib>Stellaard, Frans</creatorcontrib><creatorcontrib>Smit, G.Peter A.</creatorcontrib><creatorcontrib>Kuipers, Folkert</creatorcontrib><title>Increased lipogenesis and resistance of lipoproteins to oxidative modification in two patients with glycogen storage disease type 1a</title><title>The Journal of pediatrics</title><addtitle>J Pediatr</addtitle><description>We describe 2 patients with glycogen storage disease type 1a and severe hyperlipidemia without premature atherosclerosis. Susceptibility of low-density lipoproteins to oxidation was decreased, possibly related to the ~40-fold increase in palmitate synthesis altering lipoprotein saturated fatty acid contents. These findings are potentially relevant for antihyperlipidemic treatment in patients with glycogen storage disease type 1a. (J Pediatr 2002;140:256-60)</description><subject>Adult</subject><subject>Biological and medical sciences</subject><subject>Cholesterol, LDL - metabolism</subject><subject>Disorders of blood lipids. 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| fulltext | fulltext |
| identifier | ISSN: 0022-3476 |
| ispartof | The Journal of pediatrics, 2002-02, Vol.140 (2), p.256-260 |
| issn | 0022-3476 1097-6833 |
| language | eng |
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| source | Elsevier |
| subjects | Adult Biological and medical sciences Cholesterol, LDL - metabolism Disorders of blood lipids. Hyperlipoproteinemia Glycogen Storage Disease Type I - blood Glycogen Storage Disease Type I - complications Glycogen Storage Disease Type I - genetics Glycogen Storage Disease Type I - physiopathology Humans Hyperlipidemias - complications Lipoproteins, VLDL - metabolism Male Medical sciences Metabolic diseases Oxidation-Reduction Triglycerides - blood |
| title | Increased lipogenesis and resistance of lipoproteins to oxidative modification in two patients with glycogen storage disease type 1a |
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