Extracellular matrix turnover and disease severity in Anderson–Fabry disease
Identifieur interne : 007C69 ( Main/Exploration ); précédent : 007C68; suivant : 007C70Extracellular matrix turnover and disease severity in Anderson–Fabry disease
Auteurs : J. S. Shah [Royaume-Uni] ; D. Hughes [Royaume-Uni] ; M. Tayebjee [Royaume-Uni] ; R. Macfadyen [Royaume-Uni] ; A. Mehta [Royaume-Uni] ; P. M. Elliott [Royaume-Uni]Source :
- Acta Pædiatrica [ 0803-5253 ] ; 2006-04.
Abstract
Background: Anderson–Fabry disease (AFD) is an inherited metabolic disease associated with premature death from cardiovascular and renal disease. Recent studies have shown that patients with AFD develop progressive left‐ventricular (LV) remodelling. We hypothesized that patients with AFD have abnormal extracellular matrix (ECM) turn over compared with normal controls. Methods and Results: Twenty‐nine (mean age (±SD), 44.1±11.7 years; 15 male) consecutive patients with AFD and 21 age‐ and gender‐matched controls (mean age, 39.7±11.3 years; 10 male) had serum analysed for matrix metalloproteinase‐9 (MMP‐9), tissue inhibitor of metalloproteinases‐1 (TIMP)‐1 and ‐2 levels using an in‐house enzyme‐linked immunosorbent assay. MMP‐9 levels were significantly elevated in patients compared with controls (mean difference from controls, 427.1 ng/ml, 95% CI, 252.1 – 602.2 ng/ml, p<0.001). There was a negative correlation between MMP‐9 and fractional shortening (r = − 0.5, p=0.01). There were no differences in TIMP levels between patients and controls. There was no correlation between LV mass or maximal LV wall thickness and MMP‐9 levels. There was a positive correlation between MMP‐9 levels and the Mainz Severity Score Index (r = 0.5, p=0.01). These relationships remained significant, independently of gender and age‐using stepwise linear regression analysis. Conclusion: Patients with AFD have higher levels of MMP‐9 compared with controls. MMP‐9 levels correlated with clinical markers of disease severity suggesting that abnormal ECM turnover plays an important role in the pathogenesis of AFD. This in turn suggests that MMP‐9 levels may be a useful circulating marker for disease severity and a surrogate marker for the response to enzyme replacement therapy.
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DOI: 10.1111/j.1651-2227.2006.tb02400.x
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S." last="Shah">J. S. Shah</name><affiliation wicri:level="4"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>The Heart Hospital, University College London, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region><settlement type="city">Londres</settlement></placeName><orgName type="university">University College de Londres</orgName></affiliation></author><author><name sortKey="Hughes, D" sort="Hughes, D" uniqKey="Hughes D" first="D." last="Hughes">D. 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Macfadyen</name><affiliation wicri:level="1"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>City Hospital, Dudley Road Birmingham</wicri:regionArea><wicri:noRegion>Dudley Road Birmingham</wicri:noRegion></affiliation></author><author><name sortKey="Mehta, A" sort="Mehta, A" uniqKey="Mehta A" first="A." last="Mehta">A. Mehta</name><affiliation wicri:level="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>The Lysosomal Storage Disorders Unit, The Royal Free Hospital, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author><author><name sortKey="Elliott, P M" sort="Elliott, P M" uniqKey="Elliott P" first="P. M." last="Elliott">P. M. Elliott</name><affiliation wicri:level="4"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>The Heart Hospital, University College London, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region><settlement type="city">Londres</settlement></placeName><orgName type="university">University College de Londres</orgName></affiliation></author></analytic><monogr></monogr><series><title level="j" type="main">Acta Pædiatrica</title><title level="j" type="alt">ACTA PAEDIATRICA</title><idno type="ISSN">0803-5253</idno><idno type="eISSN">1651-2227</idno><imprint><biblScope unit="vol">95</biblScope><biblScope unit="page" from="118">118</biblScope><biblScope unit="page" to="123">123</biblScope><biblScope unit="page-count">6</biblScope><publisher>Blackwell Publishing Ltd</publisher><pubPlace>Oxford, UK</pubPlace><date type="published" when="2006-04">2006-04</date></imprint><idno type="ISSN">0803-5253</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0803-5253</idno></seriesStmt></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Background: Anderson–Fabry disease (AFD) is an inherited metabolic disease associated with premature death from cardiovascular and renal disease. Recent studies have shown that patients with AFD develop progressive left‐ventricular (LV) remodelling. We hypothesized that patients with AFD have abnormal extracellular matrix (ECM) turn over compared with normal controls. Methods and Results: Twenty‐nine (mean age (±SD), 44.1±11.7 years; 15 male) consecutive patients with AFD and 21 age‐ and gender‐matched controls (mean age, 39.7±11.3 years; 10 male) had serum analysed for matrix metalloproteinase‐9 (MMP‐9), tissue inhibitor of metalloproteinases‐1 (TIMP)‐1 and ‐2 levels using an in‐house enzyme‐linked immunosorbent assay. MMP‐9 levels were significantly elevated in patients compared with controls (mean difference from controls, 427.1 ng/ml, 95% CI, 252.1 – 602.2 ng/ml, p<0.001). There was a negative correlation between MMP‐9 and fractional shortening (r = − 0.5, p=0.01). There were no differences in TIMP levels between patients and controls. There was no correlation between LV mass or maximal LV wall thickness and MMP‐9 levels. There was a positive correlation between MMP‐9 levels and the Mainz Severity Score Index (r = 0.5, p=0.01). These relationships remained significant, independently of gender and age‐using stepwise linear regression analysis. Conclusion: Patients with AFD have higher levels of MMP‐9 compared with controls. MMP‐9 levels correlated with clinical markers of disease severity suggesting that abnormal ECM turnover plays an important role in the pathogenesis of AFD. This in turn suggests that MMP‐9 levels may be a useful circulating marker for disease severity and a surrogate marker for the response to enzyme replacement therapy.</div></front></TEI><affiliations><list><country><li>Royaume-Uni</li></country><region><li>Angleterre</li><li>Grand Londres</li></region><settlement><li>Londres</li></settlement><orgName><li>University College de Londres</li></orgName></list><tree><country name="Royaume-Uni"><region name="Angleterre"><name sortKey="Shah, J S" sort="Shah, J S" uniqKey="Shah J" first="J. S." last="Shah">J. S. Shah</name></region><name sortKey="Elliott, P M" sort="Elliott, P M" uniqKey="Elliott P" first="P. M." last="Elliott">P. M. Elliott</name><name sortKey="Hughes, D" sort="Hughes, D" uniqKey="Hughes D" first="D." last="Hughes">D. Hughes</name><name sortKey="Macfadyen, R" sort="Macfadyen, R" uniqKey="Macfadyen R" first="R." last="Macfadyen">R. Macfadyen</name><name sortKey="Mehta, A" sort="Mehta, A" uniqKey="Mehta A" first="A." last="Mehta">A. Mehta</name><name sortKey="Tayebjee, M" sort="Tayebjee, M" uniqKey="Tayebjee M" first="M." last="Tayebjee">M. Tayebjee</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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