Microfibril-associated glycoproteins MAGP-1 and MAGP-2 in disease

Microfibril-associated glycoproteins 1 and 2 (MAGP-1, MAGP-2) are protein components of extracellular matrix microfibrils. These proteins interact with fibrillin, the core component of microfibrils, and impart unique biological properties that influence microfibril function in vertebrates. MAGPs bin...

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Veröffentlicht in:Matrix biology 2018-10, Vol.71-72, p.100-111
Hauptverfasser: Craft, Clarissa S., Broekelmann, Thomas J., Mecham, Robert P.
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Mecham, Robert P.
description Microfibril-associated glycoproteins 1 and 2 (MAGP-1, MAGP-2) are protein components of extracellular matrix microfibrils. These proteins interact with fibrillin, the core component of microfibrils, and impart unique biological properties that influence microfibril function in vertebrates. MAGPs bind active forms of TGFβ and BMPs and are capable of modulating Notch signaling. Mutations in MAGP-1 or MAGP-2 have been linked to thoracic aneurysms and metabolic disease in humans. MAGP-2 has also been shown to be an important biomarker in several human cancers. Mice lacking MAGP-1 or MAGP-2 have defects in multiple organ systems, which reflects the widespread distribution of microfibrils in vertebrate tissues. This review summarizes our current understanding of the function of the MAGPs and their relationship to human disease. •MAGP-1 and MAGP-2 are microfibril-associated proteins that work with fibrillin to define microfibril function.•Both MAGPs bind active forms of TGFβ and BMPs, and are capable of modulating Notch signaling.•Neither protein is required for normal development, but MAGP gene mutations lead to defects in multiple organ systems.
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subjects Aneurysms
Animals
Aortic Aneurysm, Thoracic - genetics
Aortic Aneurysm, Thoracic - metabolism
Biomarkers
Biomarkers - metabolism
Bone
Bone Morphogenetic Proteins - metabolism
Cancer
Contractile Proteins - genetics
Contractile Proteins - metabolism
Extracellular matrix
Extracellular Matrix - metabolism
Extracellular Matrix Proteins - genetics
Extracellular Matrix Proteins - metabolism
Fibrillin
Glycoproteins
Humans
MAGP
Metabolic Diseases - genetics
Metabolic Diseases - metabolism
Metabolic disorders
Mice
Microfibril
Microfibril-associated glycoprotein
Microfibrils
Mutation
Neoplasms - genetics
Neoplasms - metabolism
Obesity
Receptors, Notch - metabolism
Signal Transduction
Thorax
Transforming Growth Factor beta - metabolism
title Microfibril-associated glycoproteins MAGP-1 and MAGP-2 in disease
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