Microfibrils: a cornerstone of extracellular matrix and a key to understand Marfan syndrome.

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Article

Microfibrils: a cornerstone of extracellular matrix and a key to understand Marfan syndrome.

Italian journal of anatomy and embryology = Archivio italiano di anatomia ed embriologia - 1 Jan 2000

Bonetti Maria Ida

Abstract excerpt

The extracellular matrix is made of collagen, reticular, elastic and oxytalan fibers, amorphous ground substance and adhesive proteins playing a structural role, such as fibronectin; the basement membrane is a specialized matrix compartment which adheres to non-connective tissues and is continuous with the remaining matrix thanks to reticular fibers, anchoring fibrils, collagen VI filaments and oxytalan fibers....

Topics

Animals
Connective Tissue
Extracellular Matrix
Fibrillin-1
Fibrillins
Genetic Predisposition to Disease
Humans
Marfan Syndrome
Microfibrils
Microfilament Proteins
Mutation
Signal Transduction
Transforming Growth Factor beta

Source

Open in PubMed

Identifiers

PMID: 20578676

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 31 Aug 2026.

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