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

Research output: Contribution to journalReview article

14 Scopus citations

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 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.

Original languageEnglish
Pages (from-to)100-111
Number of pages12
JournalMatrix Biology
Volume71-72
DOIs
StatePublished - Oct 2018

Keywords

  • Aneurysms
  • Bone
  • Cancer
  • Fibrillin
  • MAGP
  • Microfibril
  • Microfibril-associated glycoprotein
  • Obesity

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