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Article

Characterisation of fibrillin-1 cDNA clones in a human fibroblast cell line that assembles microfibrils.

The international journal of biochemistry & cell biology - 1 Feb 2000

Kettle S, Card C M, Hutchinson S, Sykes B, Handford P A

Abstract excerpt

Fibrillin-1 is a large extracellular glycoprotein which is a major structural component of 10-12 nm microfibrils. Defects in human fibrillin-1 give rise to the autosomal dominant connective tissue disease the Marfan syndrome and related disorders. Previous studies examining the biosynthesis and secretion of recombinant fibrillin-1 fragments have been performed in cell lines which do not assemble fibrillin into...

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PMID: 10687954
DOI: 10.1016/s1357-2725(99)00120-x

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

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