Mutant COMP shapes growth and development of skull and facial structures in mice and humans
Abstract Background Cartilage oligomeric matrix protein (COMP) is an important extracellular matrix protein primarily functioning in the musculoskeletal tissues and especially endochondral bone growth. Mutations in COMP cause the skeletal dysplasia pseudoachondroplasia (PSACH) that is characterized...
Ausführliche Beschreibung
Autor*in: |
Alexander Burger [verfasserIn] Jasmien Roosenboom [verfasserIn] Mohammad Hossain [verfasserIn] Seth M. Weinberg [verfasserIn] Jacqueline T. Hecht [verfasserIn] Karen L. Posey [verfasserIn] |
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E-Artikel |
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Sprache: |
Englisch |
Erschienen: |
2020 |
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In: Molecular Genetics & Genomic Medicine - Wiley, 2014, 8(2020), 7, Seite n/a-n/a |
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Übergeordnetes Werk: |
volume:8 ; year:2020 ; number:7 ; pages:n/a-n/a |
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DOI / URN: |
10.1002/mgg3.1251 |
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Katalog-ID: |
DOAJ066294851 |
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520 | |a Abstract Background Cartilage oligomeric matrix protein (COMP) is an important extracellular matrix protein primarily functioning in the musculoskeletal tissues and especially endochondral bone growth. Mutations in COMP cause the skeletal dysplasia pseudoachondroplasia (PSACH) that is characterized by short limbs and fingers, joint laxity, and abnormalities but a striking lack of skull and facial abnormalities. Methods This study examined both mice and humans to determine how mutant‐COMP affects face and skull growth. Results Mutant COMP (MT‐COMP) mice were phenotypically distinct. Snout length and skull height were diminished in MT‐COMP mouse and the face more closely resembled younger controls. Three‐dimensional facial measurements of PSACH faces showed widely spaced eyes, reduced lower facial height, and decreased nasal protrusion, which correlated with a more juvenile appearing face. Neither MT‐COMP mice nor PSACH individuals show midface hypoplasia usually associated with abnormal endochondral bone growth. MT‐COMP mice do show delayed endochondral and membranous skull ossification that normalizes with age. Conclusion Therefore, mutant‐COMP affects both endochondral and intramembranous bones of the skull resulting in a reduction of the nose and lower facial height in mice and humans, in addition to its well‐defined role in the growth plate chondrocytes. | ||
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700 | 0 | |a Jasmien Roosenboom |e verfasserin |4 aut | |
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10.1002/mgg3.1251 doi (DE-627)DOAJ066294851 (DE-599)DOAJ7b8b27043b74438f9c17333176c6a3c0 DE-627 ger DE-627 rakwb eng QH426-470 Alexander Burger verfasserin aut Mutant COMP shapes growth and development of skull and facial structures in mice and humans 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Abstract Background Cartilage oligomeric matrix protein (COMP) is an important extracellular matrix protein primarily functioning in the musculoskeletal tissues and especially endochondral bone growth. Mutations in COMP cause the skeletal dysplasia pseudoachondroplasia (PSACH) that is characterized by short limbs and fingers, joint laxity, and abnormalities but a striking lack of skull and facial abnormalities. Methods This study examined both mice and humans to determine how mutant‐COMP affects face and skull growth. Results Mutant COMP (MT‐COMP) mice were phenotypically distinct. Snout length and skull height were diminished in MT‐COMP mouse and the face more closely resembled younger controls. Three‐dimensional facial measurements of PSACH faces showed widely spaced eyes, reduced lower facial height, and decreased nasal protrusion, which correlated with a more juvenile appearing face. Neither MT‐COMP mice nor PSACH individuals show midface hypoplasia usually associated with abnormal endochondral bone growth. MT‐COMP mice do show delayed endochondral and membranous skull ossification that normalizes with age. Conclusion Therefore, mutant‐COMP affects both endochondral and intramembranous bones of the skull resulting in a reduction of the nose and lower facial height in mice and humans, in addition to its well‐defined role in the growth plate chondrocytes. dwarfism face mutant cartilage oligomeric matrix protein pseudoachondroplasia skull Genetics Jasmien Roosenboom verfasserin aut Mohammad Hossain verfasserin aut Seth M. Weinberg verfasserin aut Jacqueline T. Hecht verfasserin aut Karen L. Posey verfasserin aut In Molecular Genetics & Genomic Medicine Wiley, 2014 8(2020), 7, Seite n/a-n/a (DE-627)769222234 (DE-600)2734884-2 23249269 nnns volume:8 year:2020 number:7 pages:n/a-n/a https://doi.org/10.1002/mgg3.1251 kostenfrei https://doaj.org/article/7b8b27043b74438f9c17333176c6a3c0 kostenfrei https://doi.org/10.1002/mgg3.1251 kostenfrei https://doaj.org/toc/2324-9269 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_636 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 8 2020 7 n/a-n/a |
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10.1002/mgg3.1251 doi (DE-627)DOAJ066294851 (DE-599)DOAJ7b8b27043b74438f9c17333176c6a3c0 DE-627 ger DE-627 rakwb eng QH426-470 Alexander Burger verfasserin aut Mutant COMP shapes growth and development of skull and facial structures in mice and humans 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Abstract Background Cartilage oligomeric matrix protein (COMP) is an important extracellular matrix protein primarily functioning in the musculoskeletal tissues and especially endochondral bone growth. Mutations in COMP cause the skeletal dysplasia pseudoachondroplasia (PSACH) that is characterized by short limbs and fingers, joint laxity, and abnormalities but a striking lack of skull and facial abnormalities. Methods This study examined both mice and humans to determine how mutant‐COMP affects face and skull growth. Results Mutant COMP (MT‐COMP) mice were phenotypically distinct. Snout length and skull height were diminished in MT‐COMP mouse and the face more closely resembled younger controls. Three‐dimensional facial measurements of PSACH faces showed widely spaced eyes, reduced lower facial height, and decreased nasal protrusion, which correlated with a more juvenile appearing face. Neither MT‐COMP mice nor PSACH individuals show midface hypoplasia usually associated with abnormal endochondral bone growth. MT‐COMP mice do show delayed endochondral and membranous skull ossification that normalizes with age. Conclusion Therefore, mutant‐COMP affects both endochondral and intramembranous bones of the skull resulting in a reduction of the nose and lower facial height in mice and humans, in addition to its well‐defined role in the growth plate chondrocytes. dwarfism face mutant cartilage oligomeric matrix protein pseudoachondroplasia skull Genetics Jasmien Roosenboom verfasserin aut Mohammad Hossain verfasserin aut Seth M. Weinberg verfasserin aut Jacqueline T. Hecht verfasserin aut Karen L. Posey verfasserin aut In Molecular Genetics & Genomic Medicine Wiley, 2014 8(2020), 7, Seite n/a-n/a (DE-627)769222234 (DE-600)2734884-2 23249269 nnns volume:8 year:2020 number:7 pages:n/a-n/a https://doi.org/10.1002/mgg3.1251 kostenfrei https://doaj.org/article/7b8b27043b74438f9c17333176c6a3c0 kostenfrei https://doi.org/10.1002/mgg3.1251 kostenfrei https://doaj.org/toc/2324-9269 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_636 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 8 2020 7 n/a-n/a |
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10.1002/mgg3.1251 doi (DE-627)DOAJ066294851 (DE-599)DOAJ7b8b27043b74438f9c17333176c6a3c0 DE-627 ger DE-627 rakwb eng QH426-470 Alexander Burger verfasserin aut Mutant COMP shapes growth and development of skull and facial structures in mice and humans 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Abstract Background Cartilage oligomeric matrix protein (COMP) is an important extracellular matrix protein primarily functioning in the musculoskeletal tissues and especially endochondral bone growth. Mutations in COMP cause the skeletal dysplasia pseudoachondroplasia (PSACH) that is characterized by short limbs and fingers, joint laxity, and abnormalities but a striking lack of skull and facial abnormalities. Methods This study examined both mice and humans to determine how mutant‐COMP affects face and skull growth. Results Mutant COMP (MT‐COMP) mice were phenotypically distinct. Snout length and skull height were diminished in MT‐COMP mouse and the face more closely resembled younger controls. Three‐dimensional facial measurements of PSACH faces showed widely spaced eyes, reduced lower facial height, and decreased nasal protrusion, which correlated with a more juvenile appearing face. Neither MT‐COMP mice nor PSACH individuals show midface hypoplasia usually associated with abnormal endochondral bone growth. MT‐COMP mice do show delayed endochondral and membranous skull ossification that normalizes with age. Conclusion Therefore, mutant‐COMP affects both endochondral and intramembranous bones of the skull resulting in a reduction of the nose and lower facial height in mice and humans, in addition to its well‐defined role in the growth plate chondrocytes. dwarfism face mutant cartilage oligomeric matrix protein pseudoachondroplasia skull Genetics Jasmien Roosenboom verfasserin aut Mohammad Hossain verfasserin aut Seth M. Weinberg verfasserin aut Jacqueline T. Hecht verfasserin aut Karen L. Posey verfasserin aut In Molecular Genetics & Genomic Medicine Wiley, 2014 8(2020), 7, Seite n/a-n/a (DE-627)769222234 (DE-600)2734884-2 23249269 nnns volume:8 year:2020 number:7 pages:n/a-n/a https://doi.org/10.1002/mgg3.1251 kostenfrei https://doaj.org/article/7b8b27043b74438f9c17333176c6a3c0 kostenfrei https://doi.org/10.1002/mgg3.1251 kostenfrei https://doaj.org/toc/2324-9269 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_636 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 8 2020 7 n/a-n/a |
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10.1002/mgg3.1251 doi (DE-627)DOAJ066294851 (DE-599)DOAJ7b8b27043b74438f9c17333176c6a3c0 DE-627 ger DE-627 rakwb eng QH426-470 Alexander Burger verfasserin aut Mutant COMP shapes growth and development of skull and facial structures in mice and humans 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Abstract Background Cartilage oligomeric matrix protein (COMP) is an important extracellular matrix protein primarily functioning in the musculoskeletal tissues and especially endochondral bone growth. Mutations in COMP cause the skeletal dysplasia pseudoachondroplasia (PSACH) that is characterized by short limbs and fingers, joint laxity, and abnormalities but a striking lack of skull and facial abnormalities. Methods This study examined both mice and humans to determine how mutant‐COMP affects face and skull growth. Results Mutant COMP (MT‐COMP) mice were phenotypically distinct. Snout length and skull height were diminished in MT‐COMP mouse and the face more closely resembled younger controls. Three‐dimensional facial measurements of PSACH faces showed widely spaced eyes, reduced lower facial height, and decreased nasal protrusion, which correlated with a more juvenile appearing face. Neither MT‐COMP mice nor PSACH individuals show midface hypoplasia usually associated with abnormal endochondral bone growth. MT‐COMP mice do show delayed endochondral and membranous skull ossification that normalizes with age. Conclusion Therefore, mutant‐COMP affects both endochondral and intramembranous bones of the skull resulting in a reduction of the nose and lower facial height in mice and humans, in addition to its well‐defined role in the growth plate chondrocytes. dwarfism face mutant cartilage oligomeric matrix protein pseudoachondroplasia skull Genetics Jasmien Roosenboom verfasserin aut Mohammad Hossain verfasserin aut Seth M. Weinberg verfasserin aut Jacqueline T. Hecht verfasserin aut Karen L. Posey verfasserin aut In Molecular Genetics & Genomic Medicine Wiley, 2014 8(2020), 7, Seite n/a-n/a (DE-627)769222234 (DE-600)2734884-2 23249269 nnns volume:8 year:2020 number:7 pages:n/a-n/a https://doi.org/10.1002/mgg3.1251 kostenfrei https://doaj.org/article/7b8b27043b74438f9c17333176c6a3c0 kostenfrei https://doi.org/10.1002/mgg3.1251 kostenfrei https://doaj.org/toc/2324-9269 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_636 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 8 2020 7 n/a-n/a |
allfieldsSound |
10.1002/mgg3.1251 doi (DE-627)DOAJ066294851 (DE-599)DOAJ7b8b27043b74438f9c17333176c6a3c0 DE-627 ger DE-627 rakwb eng QH426-470 Alexander Burger verfasserin aut Mutant COMP shapes growth and development of skull and facial structures in mice and humans 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Abstract Background Cartilage oligomeric matrix protein (COMP) is an important extracellular matrix protein primarily functioning in the musculoskeletal tissues and especially endochondral bone growth. Mutations in COMP cause the skeletal dysplasia pseudoachondroplasia (PSACH) that is characterized by short limbs and fingers, joint laxity, and abnormalities but a striking lack of skull and facial abnormalities. Methods This study examined both mice and humans to determine how mutant‐COMP affects face and skull growth. Results Mutant COMP (MT‐COMP) mice were phenotypically distinct. Snout length and skull height were diminished in MT‐COMP mouse and the face more closely resembled younger controls. Three‐dimensional facial measurements of PSACH faces showed widely spaced eyes, reduced lower facial height, and decreased nasal protrusion, which correlated with a more juvenile appearing face. Neither MT‐COMP mice nor PSACH individuals show midface hypoplasia usually associated with abnormal endochondral bone growth. MT‐COMP mice do show delayed endochondral and membranous skull ossification that normalizes with age. Conclusion Therefore, mutant‐COMP affects both endochondral and intramembranous bones of the skull resulting in a reduction of the nose and lower facial height in mice and humans, in addition to its well‐defined role in the growth plate chondrocytes. dwarfism face mutant cartilage oligomeric matrix protein pseudoachondroplasia skull Genetics Jasmien Roosenboom verfasserin aut Mohammad Hossain verfasserin aut Seth M. Weinberg verfasserin aut Jacqueline T. Hecht verfasserin aut Karen L. Posey verfasserin aut In Molecular Genetics & Genomic Medicine Wiley, 2014 8(2020), 7, Seite n/a-n/a (DE-627)769222234 (DE-600)2734884-2 23249269 nnns volume:8 year:2020 number:7 pages:n/a-n/a https://doi.org/10.1002/mgg3.1251 kostenfrei https://doaj.org/article/7b8b27043b74438f9c17333176c6a3c0 kostenfrei https://doi.org/10.1002/mgg3.1251 kostenfrei https://doaj.org/toc/2324-9269 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_636 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 8 2020 7 n/a-n/a |
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mutant comp shapes growth and development of skull and facial structures in mice and humans |
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Mutant COMP shapes growth and development of skull and facial structures in mice and humans |
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Abstract Background Cartilage oligomeric matrix protein (COMP) is an important extracellular matrix protein primarily functioning in the musculoskeletal tissues and especially endochondral bone growth. Mutations in COMP cause the skeletal dysplasia pseudoachondroplasia (PSACH) that is characterized by short limbs and fingers, joint laxity, and abnormalities but a striking lack of skull and facial abnormalities. Methods This study examined both mice and humans to determine how mutant‐COMP affects face and skull growth. Results Mutant COMP (MT‐COMP) mice were phenotypically distinct. Snout length and skull height were diminished in MT‐COMP mouse and the face more closely resembled younger controls. Three‐dimensional facial measurements of PSACH faces showed widely spaced eyes, reduced lower facial height, and decreased nasal protrusion, which correlated with a more juvenile appearing face. Neither MT‐COMP mice nor PSACH individuals show midface hypoplasia usually associated with abnormal endochondral bone growth. MT‐COMP mice do show delayed endochondral and membranous skull ossification that normalizes with age. Conclusion Therefore, mutant‐COMP affects both endochondral and intramembranous bones of the skull resulting in a reduction of the nose and lower facial height in mice and humans, in addition to its well‐defined role in the growth plate chondrocytes. |
abstractGer |
Abstract Background Cartilage oligomeric matrix protein (COMP) is an important extracellular matrix protein primarily functioning in the musculoskeletal tissues and especially endochondral bone growth. Mutations in COMP cause the skeletal dysplasia pseudoachondroplasia (PSACH) that is characterized by short limbs and fingers, joint laxity, and abnormalities but a striking lack of skull and facial abnormalities. Methods This study examined both mice and humans to determine how mutant‐COMP affects face and skull growth. Results Mutant COMP (MT‐COMP) mice were phenotypically distinct. Snout length and skull height were diminished in MT‐COMP mouse and the face more closely resembled younger controls. Three‐dimensional facial measurements of PSACH faces showed widely spaced eyes, reduced lower facial height, and decreased nasal protrusion, which correlated with a more juvenile appearing face. Neither MT‐COMP mice nor PSACH individuals show midface hypoplasia usually associated with abnormal endochondral bone growth. MT‐COMP mice do show delayed endochondral and membranous skull ossification that normalizes with age. Conclusion Therefore, mutant‐COMP affects both endochondral and intramembranous bones of the skull resulting in a reduction of the nose and lower facial height in mice and humans, in addition to its well‐defined role in the growth plate chondrocytes. |
abstract_unstemmed |
Abstract Background Cartilage oligomeric matrix protein (COMP) is an important extracellular matrix protein primarily functioning in the musculoskeletal tissues and especially endochondral bone growth. Mutations in COMP cause the skeletal dysplasia pseudoachondroplasia (PSACH) that is characterized by short limbs and fingers, joint laxity, and abnormalities but a striking lack of skull and facial abnormalities. Methods This study examined both mice and humans to determine how mutant‐COMP affects face and skull growth. Results Mutant COMP (MT‐COMP) mice were phenotypically distinct. Snout length and skull height were diminished in MT‐COMP mouse and the face more closely resembled younger controls. Three‐dimensional facial measurements of PSACH faces showed widely spaced eyes, reduced lower facial height, and decreased nasal protrusion, which correlated with a more juvenile appearing face. Neither MT‐COMP mice nor PSACH individuals show midface hypoplasia usually associated with abnormal endochondral bone growth. MT‐COMP mice do show delayed endochondral and membranous skull ossification that normalizes with age. Conclusion Therefore, mutant‐COMP affects both endochondral and intramembranous bones of the skull resulting in a reduction of the nose and lower facial height in mice and humans, in addition to its well‐defined role in the growth plate chondrocytes. |
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