{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,17]],"date-time":"2026-02-17T13:13:15Z","timestamp":1771333995769,"version":"3.50.1"},"reference-count":31,"publisher":"American Physiological Society","issue":"6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American Journal of Physiology-Renal Physiology"],"published-print":{"date-parts":[[2006,6]]},"abstract":"<jats:p>The microfibrillar protein fibrillin-1 is a component of the mesangial matrix. Defects in fibrillin-1 predisposes individuals to vascular damage in Marfan syndrome, but the role of fibrillin-1 in kidney disease is unknown. We hypothesized that fibrillin-1 is involved in hypertensive or diabetic glomerular disease. DOCA-salt hypertension or streptozotocin (STZ) diabetes led to a significant increase in glomerular fibrillin-1 deposition. To test the functional role of fibrillin-1, DOCA hypertension and STZ diabetes were induced in mice homozygous for a mutation leading to a fivefold lower expression of fibrillin-1 (mgR\/mgR). Untreated male mgR\/mgR mice usually die from aortic dissection during the first 4 mo of life. All DOCA-treated mgR\/mgR mice died within 2 wk after onset of DOCA treatment. DOCA-treated heterozygous (mgR\/+) and their wild-type littermates displayed similar blood pressure levels, but albuminuria was significantly lower in mgR\/+ than in wild-type mice after DOCA treatment. Similarly, STZ diabetic mgR\/mgR and mgR\/+ developed lower albuminuria than wild-type mice despite higher blood glucose levels in mgR\/mgR and mgR\/+ compared with wild-type mice. Blood pressure, blood glucose, and albuminuria did not differ among untreated mgR\/mgR, mgR\/+, and wild-type mice, respectively. In diabetic mgR\/+ and mgR\/mgR, but not in wild-type mice, an induction of glomerular decorin expression was observed. Thus underexpression of fibrillin-1 predisposes individuals to lethal aortic dissection in the presence of hypertension. On the other hand, albuminuria as a parameter of microvascular damage in hypertension and diabetes was ameliorated in fibrillin-1-underexpressing mice, possibly due to a compensatory upregulation of decorin. We conclude that fibrillin-1 may contribute to glomerular damage in hypertensive and diabetic kidney disease.<\/jats:p>","DOI":"10.1152\/ajprenal.00284.2005","type":"journal-article","created":{"date-parts":[[2005,12,28]],"date-time":"2005-12-28T01:23:48Z","timestamp":1135733028000},"page":"F1329-F1336","source":"Crossref","is-referenced-by-count":20,"title":["Role of fibrillin-1 in hypertensive and diabetic glomerular disease"],"prefix":"10.1152","volume":"290","author":[{"given":"Andrea","family":"Hartner","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Liliana","family":"Schaefer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Markus","family":"Porst","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nada","family":"Cordasic","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anke","family":"Gabriel","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bernd","family":"Klanke","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dieter P.","family":"Reinhardt","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Karl F.","family":"Hilgers","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"24","reference":[{"key":"R1","doi-asserted-by":"publisher","DOI":"10.3109\/00365599409181282"},{"key":"R2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1523-1755.2004.66005.x"},{"key":"R3","doi-asserted-by":"publisher","DOI":"10.1038\/360361a0"},{"key":"R4","unstructured":"Cleary EG and Gibson MA.Elastic tissue, elastin and elastin associated microfibrills. 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