Prominent increase in plasma ganglioside GM3 is associated with clinical manifestations of type I Gaucher disease
Patients with Gaucher disease show signs of insulin resistance. The ganglioside GM3 has recently shown to be a negative regulator of insulin sensitivity. In fibroblasts of Gaucher patients, deficient in degradation of glucosylceramide, an increased anabolism of this lipid to gangliosides occurs. The...
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| Veröffentlicht in: | Clinica chimica acta 2008-03, Vol.389 (1-2), p.109-113 |
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| container_title | Clinica chimica acta |
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| creator | Ghauharali-van der Vlugt, Karen Langeveld, Mirjam Poppema, Aldi Kuiper, Sijmen Hollak, Carla E.M. Aerts, Johannes M. Groener, Johanna E.M. |
| description | Patients with Gaucher disease show signs of insulin resistance. The ganglioside GM3 has recently shown to be a negative regulator of insulin sensitivity. In fibroblasts of Gaucher patients, deficient in degradation of glucosylceramide, an increased anabolism of this lipid to gangliosides occurs. The goal of the current study was to establish whether GM3 is elevated in plasma of type I Gaucher disease patients, and is related to disease manifestations.
Plasma GM3, glucosylceramide, and ceramide were determined and compared to overall severity of disease, hepatomegaly, and plasma chitotriosidase activity.
The ceramide concentration in plasma of untreated Gaucher patients was slightly but not significantly lower than in controls (median: 9.8 µmol/L, range: 5.7–14.7 µmol/L, (n=40) vs. median: 11.0 µmol/L, range: 5.1–18.0 µmol/L, (n=30)). Glucosylceramide was significantly (p |
| doi_str_mv | 10.1016/j.cca.2007.12.001 |
| format | Article |
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Plasma GM3, glucosylceramide, and ceramide were determined and compared to overall severity of disease, hepatomegaly, and plasma chitotriosidase activity.
The ceramide concentration in plasma of untreated Gaucher patients was slightly but not significantly lower than in controls (median: 9.8 µmol/L, range: 5.7–14.7 µmol/L, (n=40) vs. median: 11.0 µmol/L, range: 5.1–18.0 µmol/L, (n=30)). Glucosylceramide was significantly (p<0.0001) elevated. GM3 was also significantly (p<0.0001) increased (median: 10.2 µmol/L, range: 4.3–19.1 µmol/L, (n=40) vs. median: 3.6 µmol/L, range: 2.7–5.4 µmol/L, (n=30)). Plasma GM3 concentrations correlated with those of plasma chitotriosidase activity (ρ=0.45, p=0.0036), overall severity of disease (ρ=0.39, p=0.012), and hepatomegaly (ρ=0.49, p=0.0015).
GM3 is strikingly elevated in plasma of most Gaucher patients. The increase is comparable to that of glucosylceramide, the primary storage lipid. The marked elevations in GM3 may play a role in the insulin resistance of Gaucher patients.</description><identifier>ISSN: 0009-8981</identifier><identifier>EISSN: 1873-3492</identifier><identifier>DOI: 10.1016/j.cca.2007.12.001</identifier><identifier>PMID: 18164265</identifier><language>eng</language><publisher>Netherlands: Elsevier B.V</publisher><subject>Case-Control Studies ; Chitotriosidase ; Cohort Studies ; G(M3) Ganglioside - blood ; Ganglioside ; Gaucher disease ; Gaucher Disease - blood ; Gaucher Disease - pathology ; Glucosylceramide ; Humans ; Lysosomal storage disorder</subject><ispartof>Clinica chimica acta, 2008-03, Vol.389 (1-2), p.109-113</ispartof><rights>2007 Elsevier B.V.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c351t-f7edc80afa9d4f6dca97910acd58df3eb18e6271c1906546e33a0c808bb9cbbc3</citedby><cites>FETCH-LOGICAL-c351t-f7edc80afa9d4f6dca97910acd58df3eb18e6271c1906546e33a0c808bb9cbbc3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/j.cca.2007.12.001$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3548,27923,27924,45994</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/18164265$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Ghauharali-van der Vlugt, Karen</creatorcontrib><creatorcontrib>Langeveld, Mirjam</creatorcontrib><creatorcontrib>Poppema, Aldi</creatorcontrib><creatorcontrib>Kuiper, Sijmen</creatorcontrib><creatorcontrib>Hollak, Carla E.M.</creatorcontrib><creatorcontrib>Aerts, Johannes M.</creatorcontrib><creatorcontrib>Groener, Johanna E.M.</creatorcontrib><title>Prominent increase in plasma ganglioside GM3 is associated with clinical manifestations of type I Gaucher disease</title><title>Clinica chimica acta</title><addtitle>Clin Chim Acta</addtitle><description>Patients with Gaucher disease show signs of insulin resistance. The ganglioside GM3 has recently shown to be a negative regulator of insulin sensitivity. In fibroblasts of Gaucher patients, deficient in degradation of glucosylceramide, an increased anabolism of this lipid to gangliosides occurs. The goal of the current study was to establish whether GM3 is elevated in plasma of type I Gaucher disease patients, and is related to disease manifestations.
Plasma GM3, glucosylceramide, and ceramide were determined and compared to overall severity of disease, hepatomegaly, and plasma chitotriosidase activity.
The ceramide concentration in plasma of untreated Gaucher patients was slightly but not significantly lower than in controls (median: 9.8 µmol/L, range: 5.7–14.7 µmol/L, (n=40) vs. median: 11.0 µmol/L, range: 5.1–18.0 µmol/L, (n=30)). Glucosylceramide was significantly (p<0.0001) elevated. GM3 was also significantly (p<0.0001) increased (median: 10.2 µmol/L, range: 4.3–19.1 µmol/L, (n=40) vs. median: 3.6 µmol/L, range: 2.7–5.4 µmol/L, (n=30)). Plasma GM3 concentrations correlated with those of plasma chitotriosidase activity (ρ=0.45, p=0.0036), overall severity of disease (ρ=0.39, p=0.012), and hepatomegaly (ρ=0.49, p=0.0015).
GM3 is strikingly elevated in plasma of most Gaucher patients. The increase is comparable to that of glucosylceramide, the primary storage lipid. The marked elevations in GM3 may play a role in the insulin resistance of Gaucher patients.</description><subject>Case-Control Studies</subject><subject>Chitotriosidase</subject><subject>Cohort Studies</subject><subject>G(M3) Ganglioside - blood</subject><subject>Ganglioside</subject><subject>Gaucher disease</subject><subject>Gaucher Disease - blood</subject><subject>Gaucher Disease - pathology</subject><subject>Glucosylceramide</subject><subject>Humans</subject><subject>Lysosomal storage disorder</subject><issn>0009-8981</issn><issn>1873-3492</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2008</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kD1vFDEQhi1ERI7AD6BBruh28ey3RYUicokUFAqordnxbOLTrn2x90D59_h0J9FReSw976uZR4gPoEpQ0H3elURYVkr1JVSlUvBKbGDo66JudPVabJRSuhj0AJfibUq7_G1UB2_EJQzQNVXXbsTzjxgW59mv0nmKjInzIPczpgXlI_rH2YXkLMvt91q6JDGlQA5XtvKPW58kzc47wlku6N3EacXVBZ9kmOT6smd5J7d4oCeO0rp0rH8nLiacE78_v1fi1823n9e3xf3D9u76631BdQtrMfVsaVA4obbN1FlC3WtQSLYd7FTzCAN3VQ8EWnVt03Fdo8qBYRw1jSPVV-LTqXcfw_MhL2YWl4jnGT2HQzK9qhrdt5BBOIEUQ0qRJ7OPbsH4YkCZo2ezM9mzOXo2UJnsOWc-nssP48L2X-IsNgNfTgDnE387jiaRY09sXWRajQ3uP_V_Acowj8s</recordid><startdate>200803</startdate><enddate>200803</enddate><creator>Ghauharali-van der Vlugt, Karen</creator><creator>Langeveld, Mirjam</creator><creator>Poppema, Aldi</creator><creator>Kuiper, Sijmen</creator><creator>Hollak, Carla E.M.</creator><creator>Aerts, Johannes M.</creator><creator>Groener, Johanna E.M.</creator><general>Elsevier B.V</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>200803</creationdate><title>Prominent increase in plasma ganglioside GM3 is associated with clinical manifestations of type I Gaucher disease</title><author>Ghauharali-van der Vlugt, Karen ; Langeveld, Mirjam ; Poppema, Aldi ; Kuiper, Sijmen ; Hollak, Carla E.M. ; Aerts, Johannes M. ; Groener, Johanna E.M.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c351t-f7edc80afa9d4f6dca97910acd58df3eb18e6271c1906546e33a0c808bb9cbbc3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2008</creationdate><topic>Case-Control Studies</topic><topic>Chitotriosidase</topic><topic>Cohort Studies</topic><topic>G(M3) Ganglioside - blood</topic><topic>Ganglioside</topic><topic>Gaucher disease</topic><topic>Gaucher Disease - blood</topic><topic>Gaucher Disease - pathology</topic><topic>Glucosylceramide</topic><topic>Humans</topic><topic>Lysosomal storage disorder</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Ghauharali-van der Vlugt, Karen</creatorcontrib><creatorcontrib>Langeveld, Mirjam</creatorcontrib><creatorcontrib>Poppema, Aldi</creatorcontrib><creatorcontrib>Kuiper, Sijmen</creatorcontrib><creatorcontrib>Hollak, Carla E.M.</creatorcontrib><creatorcontrib>Aerts, Johannes M.</creatorcontrib><creatorcontrib>Groener, Johanna E.M.</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Clinica chimica acta</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Ghauharali-van der Vlugt, Karen</au><au>Langeveld, Mirjam</au><au>Poppema, Aldi</au><au>Kuiper, Sijmen</au><au>Hollak, Carla E.M.</au><au>Aerts, Johannes M.</au><au>Groener, Johanna E.M.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Prominent increase in plasma ganglioside GM3 is associated with clinical manifestations of type I Gaucher disease</atitle><jtitle>Clinica chimica acta</jtitle><addtitle>Clin Chim Acta</addtitle><date>2008-03</date><risdate>2008</risdate><volume>389</volume><issue>1-2</issue><spage>109</spage><epage>113</epage><pages>109-113</pages><issn>0009-8981</issn><eissn>1873-3492</eissn><abstract>Patients with Gaucher disease show signs of insulin resistance. The ganglioside GM3 has recently shown to be a negative regulator of insulin sensitivity. In fibroblasts of Gaucher patients, deficient in degradation of glucosylceramide, an increased anabolism of this lipid to gangliosides occurs. The goal of the current study was to establish whether GM3 is elevated in plasma of type I Gaucher disease patients, and is related to disease manifestations.
Plasma GM3, glucosylceramide, and ceramide were determined and compared to overall severity of disease, hepatomegaly, and plasma chitotriosidase activity.
The ceramide concentration in plasma of untreated Gaucher patients was slightly but not significantly lower than in controls (median: 9.8 µmol/L, range: 5.7–14.7 µmol/L, (n=40) vs. median: 11.0 µmol/L, range: 5.1–18.0 µmol/L, (n=30)). Glucosylceramide was significantly (p<0.0001) elevated. GM3 was also significantly (p<0.0001) increased (median: 10.2 µmol/L, range: 4.3–19.1 µmol/L, (n=40) vs. median: 3.6 µmol/L, range: 2.7–5.4 µmol/L, (n=30)). Plasma GM3 concentrations correlated with those of plasma chitotriosidase activity (ρ=0.45, p=0.0036), overall severity of disease (ρ=0.39, p=0.012), and hepatomegaly (ρ=0.49, p=0.0015).
GM3 is strikingly elevated in plasma of most Gaucher patients. The increase is comparable to that of glucosylceramide, the primary storage lipid. The marked elevations in GM3 may play a role in the insulin resistance of Gaucher patients.</abstract><cop>Netherlands</cop><pub>Elsevier B.V</pub><pmid>18164265</pmid><doi>10.1016/j.cca.2007.12.001</doi><tpages>5</tpages></addata></record> |
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| subjects | Case-Control Studies Chitotriosidase Cohort Studies G(M3) Ganglioside - blood Ganglioside Gaucher disease Gaucher Disease - blood Gaucher Disease - pathology Glucosylceramide Humans Lysosomal storage disorder |
| title | Prominent increase in plasma ganglioside GM3 is associated with clinical manifestations of type I Gaucher disease |
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