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 |
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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. |
doi_str_mv | 10.1016/j.matbio.2018.03.006 |
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•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.</description><identifier>ISSN: 0945-053X</identifier><identifier>EISSN: 1569-1802</identifier><identifier>DOI: 10.1016/j.matbio.2018.03.006</identifier><identifier>PMID: 29524629</identifier><language>eng</language><publisher>Netherlands: Elsevier B.V</publisher><subject>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</subject><ispartof>Matrix biology, 2018-10, Vol.71-72, p.100-111</ispartof><rights>2018 Elsevier B.V.</rights><rights>Copyright © 2018 Elsevier B.V. All rights reserved.</rights><rights>Copyright Elsevier Science Ltd. Oct 2018</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c491t-40eaa63d1425876f921e88bcdc49e39b458158535c47582fd6683afbe45dae393</citedby><cites>FETCH-LOGICAL-c491t-40eaa63d1425876f921e88bcdc49e39b458158535c47582fd6683afbe45dae393</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://www.sciencedirect.com/science/article/pii/S0945053X17304936$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>230,314,776,780,881,3537,27901,27902,65306</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/29524629$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Craft, Clarissa S.</creatorcontrib><creatorcontrib>Broekelmann, Thomas J.</creatorcontrib><creatorcontrib>Mecham, Robert P.</creatorcontrib><title>Microfibril-associated glycoproteins MAGP-1 and MAGP-2 in disease</title><title>Matrix biology</title><addtitle>Matrix Biol</addtitle><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.</description><subject>Aneurysms</subject><subject>Animals</subject><subject>Aortic Aneurysm, Thoracic - genetics</subject><subject>Aortic Aneurysm, Thoracic - metabolism</subject><subject>Biomarkers</subject><subject>Biomarkers - metabolism</subject><subject>Bone</subject><subject>Bone Morphogenetic Proteins - metabolism</subject><subject>Cancer</subject><subject>Contractile Proteins - genetics</subject><subject>Contractile Proteins - metabolism</subject><subject>Extracellular matrix</subject><subject>Extracellular Matrix - metabolism</subject><subject>Extracellular Matrix Proteins - genetics</subject><subject>Extracellular Matrix Proteins - metabolism</subject><subject>Fibrillin</subject><subject>Glycoproteins</subject><subject>Humans</subject><subject>MAGP</subject><subject>Metabolic Diseases - genetics</subject><subject>Metabolic Diseases - metabolism</subject><subject>Metabolic disorders</subject><subject>Mice</subject><subject>Microfibril</subject><subject>Microfibril-associated glycoprotein</subject><subject>Microfibrils</subject><subject>Mutation</subject><subject>Neoplasms - genetics</subject><subject>Neoplasms - metabolism</subject><subject>Obesity</subject><subject>Receptors, Notch - metabolism</subject><subject>Signal Transduction</subject><subject>Thorax</subject><subject>Transforming Growth Factor beta - metabolism</subject><issn>0945-053X</issn><issn>1569-1802</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2018</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kUtr3DAUhUVoSSZJ_0Ephm66sStdPSxvCkPICxLaRQvdCVm6TjV4rKnkCeTfV8Pk0XTRlQT3u0c65xDyntGGUaY-r5q1nfsQG6BMN5Q3lKoDsmBSdTXTFN6QBe2ErKnkP4_Icc4rSqkQrT4kR9BJEAq6BVneBpfiEPoUxtrmHF2wM_rqbnxwcZPijGHK1e3y8lvNKjv5_RWqMFU-ZLQZT8nbwY4Z3z2eJ-THxfn3s6v65uvl9dnypnaiY3MtKFqruGcCpG7V0AFDrXvnyxh51wupmdSSSydaqWHwSmluhx6F9LYA_IR82etutv0avcNpTnY0mxTWNj2YaIN5PZnCL3MX741ioFutisCnR4EUf28xz2YdssNxtBPGbTYlR-iAM9ihH_9BV3GbpmLPAOMAoDltCyX2VEkw54TD82cYNbuOzMrsO9ppa0O5KR2VtQ9_G3leeirlxSmWOO8DJpNdwMmhDwndbHwM_3_hDzFLowE</recordid><startdate>20181001</startdate><enddate>20181001</enddate><creator>Craft, Clarissa S.</creator><creator>Broekelmann, Thomas J.</creator><creator>Mecham, Robert P.</creator><general>Elsevier B.V</general><general>Elsevier Science Ltd</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>20181001</creationdate><title>Microfibril-associated glycoproteins MAGP-1 and MAGP-2 in disease</title><author>Craft, Clarissa S. ; Broekelmann, Thomas J. ; Mecham, Robert P.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c491t-40eaa63d1425876f921e88bcdc49e39b458158535c47582fd6683afbe45dae393</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2018</creationdate><topic>Aneurysms</topic><topic>Animals</topic><topic>Aortic Aneurysm, Thoracic - genetics</topic><topic>Aortic Aneurysm, Thoracic - metabolism</topic><topic>Biomarkers</topic><topic>Biomarkers - metabolism</topic><topic>Bone</topic><topic>Bone Morphogenetic Proteins - metabolism</topic><topic>Cancer</topic><topic>Contractile Proteins - genetics</topic><topic>Contractile Proteins - metabolism</topic><topic>Extracellular matrix</topic><topic>Extracellular Matrix - metabolism</topic><topic>Extracellular Matrix Proteins - genetics</topic><topic>Extracellular Matrix Proteins - metabolism</topic><topic>Fibrillin</topic><topic>Glycoproteins</topic><topic>Humans</topic><topic>MAGP</topic><topic>Metabolic Diseases - genetics</topic><topic>Metabolic Diseases - metabolism</topic><topic>Metabolic disorders</topic><topic>Mice</topic><topic>Microfibril</topic><topic>Microfibril-associated glycoprotein</topic><topic>Microfibrils</topic><topic>Mutation</topic><topic>Neoplasms - genetics</topic><topic>Neoplasms - metabolism</topic><topic>Obesity</topic><topic>Receptors, Notch - metabolism</topic><topic>Signal Transduction</topic><topic>Thorax</topic><topic>Transforming Growth Factor beta - metabolism</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Craft, Clarissa S.</creatorcontrib><creatorcontrib>Broekelmann, Thomas J.</creatorcontrib><creatorcontrib>Mecham, Robert P.</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Matrix biology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Craft, Clarissa S.</au><au>Broekelmann, Thomas J.</au><au>Mecham, Robert P.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Microfibril-associated glycoproteins MAGP-1 and MAGP-2 in disease</atitle><jtitle>Matrix biology</jtitle><addtitle>Matrix Biol</addtitle><date>2018-10-01</date><risdate>2018</risdate><volume>71-72</volume><spage>100</spage><epage>111</epage><pages>100-111</pages><issn>0945-053X</issn><eissn>1569-1802</eissn><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.
•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.</abstract><cop>Netherlands</cop><pub>Elsevier B.V</pub><pmid>29524629</pmid><doi>10.1016/j.matbio.2018.03.006</doi><tpages>12</tpages><oa>free_for_read</oa></addata></record> |
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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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