Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Fibrillin in Marfan syndrome and tight skin mice provides new insights into transforming growth factor-beta regulation and systemic sclerosis.

Current opinion in rheumatology - 1 Nov 2006

Lemaire Raphael, Bayle Julie, Lafyatis Robert

Abstract excerpt

PURPOSE OF REVIEW: Important recent understandings of fibrillins and fibrillin-associated microfibril proteins suggest new ways these proteins might contribute to tissue fibrosis seen in systemic sclerosis by regulating latent transforming growth factor-beta. This review discusses mutant-fibrilli...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Extracellular Matrix Proteins
Share to Fibrillin-1
Share to Fibrillins

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Marfan Syndrome

Share to Microfibrils

Share to Microfilament Proteins

Share to Scleroderma, Systemic

Share to Transforming Growth Factor beta

Source

Identifiers

PMID: 17053502
DOI: 10.1097/01.bor.0000245719.64393.57

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 2 Sept 2026. Prepared record publication-17053502-2026-09-02.2. Manifest publication-graph-cohort-2026-09-02.2.

Source receipt: PubMed via Helena Literature Corpus, retrieved 2 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Fibrillin in Marfan syndrome and tight skin mice provides new insights into transforming growth factor-beta regulation and systemic sclerosis.PMID 17053502Read ↗

Mutant fibrillin 1 from tight skin mice increases extracellular matrix incorporation of microfibril-associated glycoprotein 2 and type I collagen.Semantic proximity Read ↗

Marfan syndrome; A connective tissue disease at the crossroads of mechanotransduction, TGFβ signaling and cell stemness.Semantic proximity Read ↗

Fibrillin assemblies: extracellular determinants of tissue formation and fibrosisSemantic proximity Read ↗

Microenvironmental Regulation by Fibrillin-1Semantic proximity Read ↗

Recent progress towards a molecular understanding of Marfan syndrome.Semantic proximity Read ↗

Mutations in fibrillin-1 cause congenital scleroderma: stiff skin syndrome.Semantic proximity Read ↗

The fibrillin-Marfan syndrome connection.Semantic proximity Read ↗

Fibrillin‐rich microfibrils: Structural determinants of morphogenetic and homeostatic eventsSemantic proximity Read ↗

Marfan syndrome: from gene to therapy.Semantic proximity Read ↗

Marfan syndrome: new clues to genotype-phenotype correlations.Semantic proximity Read ↗

Structure and function of the mammalian fibrillin gene family: implications for human connective tissue diseases.Semantic proximity Read ↗

Fibrillin-containing microfibrils: structure and function in health and disease.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.