Matches in UGent Biblio for { <https://biblio.ugent.be/publication/1013923#aggregation> ?p ?o. }
Showing items 1 to 48 of
48
with 100 items per page.
- aggregation classification "A1".
- aggregation creator B604918.
- aggregation creator B604919.
- aggregation creator B604920.
- aggregation creator B604921.
- aggregation creator B604922.
- aggregation creator B604923.
- aggregation creator B604924.
- aggregation creator B604925.
- aggregation creator B604926.
- aggregation creator B604927.
- aggregation creator B604928.
- aggregation creator B604929.
- aggregation creator B604930.
- aggregation creator B604931.
- aggregation creator B604932.
- aggregation creator person.
- aggregation creator person.
- aggregation creator person.
- aggregation date "2010".
- aggregation format "application/pdf".
- aggregation hasFormat 1013923.bibtex.
- aggregation hasFormat 1013923.csv.
- aggregation hasFormat 1013923.dc.
- aggregation hasFormat 1013923.didl.
- aggregation hasFormat 1013923.doc.
- aggregation hasFormat 1013923.json.
- aggregation hasFormat 1013923.mets.
- aggregation hasFormat 1013923.mods.
- aggregation hasFormat 1013923.rdf.
- aggregation hasFormat 1013923.ris.
- aggregation hasFormat 1013923.txt.
- aggregation hasFormat 1013923.xls.
- aggregation hasFormat 1013923.yaml.
- aggregation isPartOf urn:issn:1946-6234.
- aggregation language "eng".
- aggregation publisher "AMER ASSOC ADVANCEMENT SCIENCE".
- aggregation rights "I have transferred the copyright for this publication to the publisher".
- aggregation subject "Medicine and Health Sciences".
- aggregation title "Mutations in Fibrillin-1 Cause Congenital Scleroderma: Stiff Skin Syndrome".
- aggregation abstract "The predisposition for scleroderma, defined as fibrosis and hardening of the skin, is poorly understood. We report that stiff skin syndrome (SSS), an autosomal dominant congenital form of scleroderma, is caused by mutations in the sole Arg-Gly-Asp sequence-encoding domain of fibrillin-1 that mediates integrin binding. Ordered polymers of fibrillin-1 (termed microfibrils) initiate elastic fiber assembly and bind to and regulate the activation of the profibrotic cytokine transforming growth factor-beta (TGF beta). Altered cell-matrix interactions in SSS accompany excessive microfibrillar deposition, impaired elastogenesis, and increased TGF beta concentration and signaling in the dermis. The observation of similar findings in systemic sclerosis, a more common acquired form of scleroderma, suggests broad pathogenic relevance.".
- aggregation authorList BK960540.
- aggregation issue "23".
- aggregation volume "2".
- aggregation aggregates 1016220.
- aggregation isDescribedBy 1013923.
- aggregation similarTo scitranslmed.3000488.
- aggregation similarTo LU-1013923.