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Article

Genetic and functional linkage between ADAMTS superfamily proteins and fibrillin-1: a novel mechanism influencing microfibril assembly and function.

Cellular and molecular life sciences : CMLS - 1 Oct 2011

Hubmacher Dirk, Apte Suneel S

Abstract excerpt

Tissue microfibrils contain fibrillin-1 as a major constituent. Microfibrils regulate bioavailability of TGFβ superfamily growth factors and are structurally crucial in the ocular zonule. FBN1 mutations typically cause the Marfan syndrome, an autosomal dominant disorder manifesting with skeletal overgrowth, aortic aneurysm, and lens dislocation (ectopia lentis). Infrequently, FBN1 mutations cause dominantly...

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Source

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Identifiers

PMID: 21858451
DOI: 10.1007/s00018-011-0780-9
PMCID: PMC4729447

Genes

ADAMTSL2
ADAMTS10
ADAMTS17
FBN1
ADAMTSL4

Provenance

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

Source receipt: pubmed, retrieved 7 Sept 2026.

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