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


ABSTRACT: 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 TGF? 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.

SUBMITTER: Craft CS 

PROVIDER: S-EPMC6128786 | biostudies-literature | 2018 Oct

REPOSITORIES: biostudies-literature

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

Craft Clarissa S CS   Broekelmann Thomas J TJ   Mecham Robert P RP  

Matrix biology : journal of the International Society for Matrix Biology 20180307


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 bee  ...[more]

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