Unraveling the Role of TGF β Signaling in Thoracic Aortic Aneurysm and Dissection Using Fbn1 Mutant Mouse Models
Matrix Biol. 2023 Sep 6:S0945-053X(23)00095-1. doi: 10.1016/j.matbio.2023.09.001. Online ahead of print.ABSTRACTAlthough abnormal TGFβ signaling is observed in several heritable forms of thoracic aortic aneurysms and dissections including Marfan syndrome, its precise role in aortic disease progression is still disputed. Using a mouse genetic approach and quantitative isobaric labeling proteomics, we sought to elucidate the role of TGFβ signaling in three Fbn1 mutant mouse models representing a range of aortic disease from microdissection (without aneurysm) to aneurysm (without rupture) to aneurysm and rupture. Results in...
Source: Matrix Biology - September 8, 2023 Category: Molecular Biology Authors: Violette Deleeuw Eric Carlson Marjolijn Renard Keith D Zientek Phillip A Wilmarth Ashok P Reddy Elise C Manalo Sara F Tufa Douglas R Keene Margie Olbinado Marco Stampanoni Sachiko Kanki Hiromi Yanagisawa Laura Mui ño Mosquera Patrick Sips Julie De Backer Source Type: research
Unraveling the Role of TGF β Signaling in Thoracic Aortic Aneurysm and Dissection Using Fbn1 Mutant Mouse Models
Matrix Biol. 2023 Sep 6:S0945-053X(23)00095-1. doi: 10.1016/j.matbio.2023.09.001. Online ahead of print.ABSTRACTAlthough abnormal TGFβ signaling is observed in several heritable forms of thoracic aortic aneurysms and dissections including Marfan syndrome, its precise role in aortic disease progression is still disputed. Using a mouse genetic approach and quantitative isobaric labeling proteomics, we sought to elucidate the role of TGFβ signaling in three Fbn1 mutant mouse models representing a range of aortic disease from microdissection (without aneurysm) to aneurysm (without rupture) to aneurysm and rupture. Results in...
Source: Matrix Biology - September 8, 2023 Category: Molecular Biology Authors: Violette Deleeuw Eric Carlson Marjolijn Renard Keith D Zientek Phillip A Wilmarth Ashok P Reddy Elise C Manalo Sara F Tufa Douglas R Keene Margie Olbinado Marco Stampanoni Sachiko Kanki Hiromi Yanagisawa Laura Mui ño Mosquera Patrick Sips Julie De Backer Source Type: research
Unraveling the Role of TGF β Signaling in Thoracic Aortic Aneurysm and Dissection Using Fbn1 Mutant Mouse Models
Matrix Biol. 2023 Sep 6:S0945-053X(23)00095-1. doi: 10.1016/j.matbio.2023.09.001. Online ahead of print.ABSTRACTAlthough abnormal TGFβ signaling is observed in several heritable forms of thoracic aortic aneurysms and dissections including Marfan syndrome, its precise role in aortic disease progression is still disputed. Using a mouse genetic approach and quantitative isobaric labeling proteomics, we sought to elucidate the role of TGFβ signaling in three Fbn1 mutant mouse models representing a range of aortic disease from microdissection (without aneurysm) to aneurysm (without rupture) to aneurysm and rupture. Results in...
Source: Matrix Biology - September 8, 2023 Category: Molecular Biology Authors: Violette Deleeuw Eric Carlson Marjolijn Renard Keith D Zientek Phillip A Wilmarth Ashok P Reddy Elise C Manalo Sara F Tufa Douglas R Keene Margie Olbinado Marco Stampanoni Sachiko Kanki Hiromi Yanagisawa Laura Mui ño Mosquera Patrick Sips Julie De Backer Source Type: research
Unraveling the Role of TGF β Signaling in Thoracic Aortic Aneurysm and Dissection Using Fbn1 Mutant Mouse Models
Matrix Biol. 2023 Sep 6:S0945-053X(23)00095-1. doi: 10.1016/j.matbio.2023.09.001. Online ahead of print.ABSTRACTAlthough abnormal TGFβ signaling is observed in several heritable forms of thoracic aortic aneurysms and dissections including Marfan syndrome, its precise role in aortic disease progression is still disputed. Using a mouse genetic approach and quantitative isobaric labeling proteomics, we sought to elucidate the role of TGFβ signaling in three Fbn1 mutant mouse models representing a range of aortic disease from microdissection (without aneurysm) to aneurysm (without rupture) to aneurysm and rupture. Results in...
Source: Matrix Biology - September 8, 2023 Category: Molecular Biology Authors: Violette Deleeuw Eric Carlson Marjolijn Renard Keith D Zientek Phillip A Wilmarth Ashok P Reddy Elise C Manalo Sara F Tufa Douglas R Keene Margie Olbinado Marco Stampanoni Sachiko Kanki Hiromi Yanagisawa Laura Mui ño Mosquera Patrick Sips Julie De Backer Source Type: research
Unraveling the Role of TGF β Signaling in Thoracic Aortic Aneurysm and Dissection Using Fbn1 Mutant Mouse Models
Matrix Biol. 2023 Sep 6:S0945-053X(23)00095-1. doi: 10.1016/j.matbio.2023.09.001. Online ahead of print.ABSTRACTAlthough abnormal TGFβ signaling is observed in several heritable forms of thoracic aortic aneurysms and dissections including Marfan syndrome, its precise role in aortic disease progression is still disputed. Using a mouse genetic approach and quantitative isobaric labeling proteomics, we sought to elucidate the role of TGFβ signaling in three Fbn1 mutant mouse models representing a range of aortic disease from microdissection (without aneurysm) to aneurysm (without rupture) to aneurysm and rupture. Results in...
Source: Matrix Biology - September 8, 2023 Category: Molecular Biology Authors: Violette Deleeuw Eric Carlson Marjolijn Renard Keith D Zientek Phillip A Wilmarth Ashok P Reddy Elise C Manalo Sara F Tufa Douglas R Keene Margie Olbinado Marco Stampanoni Sachiko Kanki Hiromi Yanagisawa Laura Mui ño Mosquera Patrick Sips Julie De Backer Source Type: research
Unraveling the Role of TGF β Signaling in Thoracic Aortic Aneurysm and Dissection Using Fbn1 Mutant Mouse Models
Matrix Biol. 2023 Sep 6:S0945-053X(23)00095-1. doi: 10.1016/j.matbio.2023.09.001. Online ahead of print.ABSTRACTAlthough abnormal TGFβ signaling is observed in several heritable forms of thoracic aortic aneurysms and dissections including Marfan syndrome, its precise role in aortic disease progression is still disputed. Using a mouse genetic approach and quantitative isobaric labeling proteomics, we sought to elucidate the role of TGFβ signaling in three Fbn1 mutant mouse models representing a range of aortic disease from microdissection (without aneurysm) to aneurysm (without rupture) to aneurysm and rupture. Results in...
Source: Matrix Biology - September 8, 2023 Category: Molecular Biology Authors: Violette Deleeuw Eric Carlson Marjolijn Renard Keith D Zientek Phillip A Wilmarth Ashok P Reddy Elise C Manalo Sara F Tufa Douglas R Keene Margie Olbinado Marco Stampanoni Sachiko Kanki Hiromi Yanagisawa Laura Mui ño Mosquera Patrick Sips Julie De Backer Source Type: research
Unraveling the Role of TGF β Signaling in Thoracic Aortic Aneurysm and Dissection Using Fbn1 Mutant Mouse Models
Matrix Biol. 2023 Sep 6:S0945-053X(23)00095-1. doi: 10.1016/j.matbio.2023.09.001. Online ahead of print.ABSTRACTAlthough abnormal TGFβ signaling is observed in several heritable forms of thoracic aortic aneurysms and dissections including Marfan syndrome, its precise role in aortic disease progression is still disputed. Using a mouse genetic approach and quantitative isobaric labeling proteomics, we sought to elucidate the role of TGFβ signaling in three Fbn1 mutant mouse models representing a range of aortic disease from microdissection (without aneurysm) to aneurysm (without rupture) to aneurysm and rupture. Results in...
Source: Matrix Biology - September 8, 2023 Category: Molecular Biology Authors: Violette Deleeuw Eric Carlson Marjolijn Renard Keith D Zientek Phillip A Wilmarth Ashok P Reddy Elise C Manalo Sara F Tufa Douglas R Keene Margie Olbinado Marco Stampanoni Sachiko Kanki Hiromi Yanagisawa Laura Mui ño Mosquera Patrick Sips Julie De Backer Source Type: research
Unraveling the Role of TGF β Signaling in Thoracic Aortic Aneurysm and Dissection Using Fbn1 Mutant Mouse Models
Matrix Biol. 2023 Sep 6:S0945-053X(23)00095-1. doi: 10.1016/j.matbio.2023.09.001. Online ahead of print.ABSTRACTAlthough abnormal TGFβ signaling is observed in several heritable forms of thoracic aortic aneurysms and dissections including Marfan syndrome, its precise role in aortic disease progression is still disputed. Using a mouse genetic approach and quantitative isobaric labeling proteomics, we sought to elucidate the role of TGFβ signaling in three Fbn1 mutant mouse models representing a range of aortic disease from microdissection (without aneurysm) to aneurysm (without rupture) to aneurysm and rupture. Results in...
Source: Matrix Biology - September 8, 2023 Category: Molecular Biology Authors: Violette Deleeuw Eric Carlson Marjolijn Renard Keith D Zientek Phillip A Wilmarth Ashok P Reddy Elise C Manalo Sara F Tufa Douglas R Keene Margie Olbinado Marco Stampanoni Sachiko Kanki Hiromi Yanagisawa Laura Mui ño Mosquera Patrick Sips Julie De Backer Source Type: research
Unraveling the Role of TGF β Signaling in Thoracic Aortic Aneurysm and Dissection Using Fbn1 Mutant Mouse Models
Matrix Biol. 2023 Sep 6:S0945-053X(23)00095-1. doi: 10.1016/j.matbio.2023.09.001. Online ahead of print.ABSTRACTAlthough abnormal TGFβ signaling is observed in several heritable forms of thoracic aortic aneurysms and dissections including Marfan syndrome, its precise role in aortic disease progression is still disputed. Using a mouse genetic approach and quantitative isobaric labeling proteomics, we sought to elucidate the role of TGFβ signaling in three Fbn1 mutant mouse models representing a range of aortic disease from microdissection (without aneurysm) to aneurysm (without rupture) to aneurysm and rupture. Results in...
Source: Matrix Biology - September 8, 2023 Category: Molecular Biology Authors: Violette Deleeuw Eric Carlson Marjolijn Renard Keith D Zientek Phillip A Wilmarth Ashok P Reddy Elise C Manalo Sara F Tufa Douglas R Keene Margie Olbinado Marco Stampanoni Sachiko Kanki Hiromi Yanagisawa Laura Mui ño Mosquera Patrick Sips Julie De Backer Source Type: research
Unraveling the Role of TGF β Signaling in Thoracic Aortic Aneurysm and Dissection Using Fbn1 Mutant Mouse Models
Matrix Biol. 2023 Sep 6:S0945-053X(23)00095-1. doi: 10.1016/j.matbio.2023.09.001. Online ahead of print.ABSTRACTAlthough abnormal TGFβ signaling is observed in several heritable forms of thoracic aortic aneurysms and dissections including Marfan syndrome, its precise role in aortic disease progression is still disputed. Using a mouse genetic approach and quantitative isobaric labeling proteomics, we sought to elucidate the role of TGFβ signaling in three Fbn1 mutant mouse models representing a range of aortic disease from microdissection (without aneurysm) to aneurysm (without rupture) to aneurysm and rupture. Results in...
Source: Matrix Biology - September 8, 2023 Category: Molecular Biology Authors: Violette Deleeuw Eric Carlson Marjolijn Renard Keith D Zientek Phillip A Wilmarth Ashok P Reddy Elise C Manalo Sara F Tufa Douglas R Keene Margie Olbinado Marco Stampanoni Sachiko Kanki Hiromi Yanagisawa Laura Mui ño Mosquera Patrick Sips Julie De Backer Source Type: research
Autocrine IL-6 drives cell and extracellular matrix anisotropy in scar fibroblasts
Matrix Biol. 2023 Sep 1:S0945-053X(23)00091-4. doi: 10.1016/j.matbio.2023.08.004. Online ahead of print.ABSTRACTFibrosis is associated with dramatic changes in extracellular matrix (ECM) architecture of unknown aetiology. Here we exploit keloid scars as a paradigm to understand fibrotic ECM organization. We reveal that keloid patient fibroblasts uniquely produce a globally aligned ECM network in 2-D culture as observed in scar tissue. ECM anisotropy develops after rapid initiation of a fibroblast supracellular actin network, suggesting that cell alignment initiates ECM patterning. Keloid fibroblasts produce elevated levels...
Source: Matrix Biology - September 3, 2023 Category: Molecular Biology Authors: Fiona N Kenny Stefania Marcotti Deandra Belo De Freitas Elena M Drudi Vivienne Leech Rachel E Bell Jennifer Easton Mar ía-Del-Carmen Díaz-de-la-Loza Roland Fleck Leanne Allison Christina Philippeos Angelika Manhart Tanya Shaw Brian M Stramer Source Type: research
Autocrine IL-6 drives cell and extracellular matrix anisotropy in scar fibroblasts
Matrix Biol. 2023 Sep 1:S0945-053X(23)00091-4. doi: 10.1016/j.matbio.2023.08.004. Online ahead of print.ABSTRACTFibrosis is associated with dramatic changes in extracellular matrix (ECM) architecture of unknown aetiology. Here we exploit keloid scars as a paradigm to understand fibrotic ECM organization. We reveal that keloid patient fibroblasts uniquely produce a globally aligned ECM network in 2-D culture as observed in scar tissue. ECM anisotropy develops after rapid initiation of a fibroblast supracellular actin network, suggesting that cell alignment initiates ECM patterning. Keloid fibroblasts produce elevated levels...
Source: Matrix Biology - September 3, 2023 Category: Molecular Biology Authors: Fiona N Kenny Stefania Marcotti Deandra Belo De Freitas Elena M Drudi Vivienne Leech Rachel E Bell Jennifer Easton Mar ía-Del-Carmen Díaz-de-la-Loza Roland Fleck Leanne Allison Christina Philippeos Angelika Manhart Tanya Shaw Brian M Stramer Source Type: research
Autocrine IL-6 drives cell and extracellular matrix anisotropy in scar fibroblasts
Matrix Biol. 2023 Sep 1:S0945-053X(23)00091-4. doi: 10.1016/j.matbio.2023.08.004. Online ahead of print.ABSTRACTFibrosis is associated with dramatic changes in extracellular matrix (ECM) architecture of unknown aetiology. Here we exploit keloid scars as a paradigm to understand fibrotic ECM organization. We reveal that keloid patient fibroblasts uniquely produce a globally aligned ECM network in 2-D culture as observed in scar tissue. ECM anisotropy develops after rapid initiation of a fibroblast supracellular actin network, suggesting that cell alignment initiates ECM patterning. Keloid fibroblasts produce elevated levels...
Source: Matrix Biology - September 3, 2023 Category: Molecular Biology Authors: Fiona N Kenny Stefania Marcotti Deandra Belo De Freitas Elena M Drudi Vivienne Leech Rachel E Bell Jennifer Easton Mar ía-Del-Carmen Díaz-de-la-Loza Roland Fleck Leanne Allison Christina Philippeos Angelika Manhart Tanya Shaw Brian M Stramer Source Type: research
Autocrine IL-6 drives cell and extracellular matrix anisotropy in scar fibroblasts
Matrix Biol. 2023 Sep 1:S0945-053X(23)00091-4. doi: 10.1016/j.matbio.2023.08.004. Online ahead of print.ABSTRACTFibrosis is associated with dramatic changes in extracellular matrix (ECM) architecture of unknown aetiology. Here we exploit keloid scars as a paradigm to understand fibrotic ECM organization. We reveal that keloid patient fibroblasts uniquely produce a globally aligned ECM network in 2-D culture as observed in scar tissue. ECM anisotropy develops after rapid initiation of a fibroblast supracellular actin network, suggesting that cell alignment initiates ECM patterning. Keloid fibroblasts produce elevated levels...
Source: Matrix Biology - September 3, 2023 Category: Molecular Biology Authors: Fiona N Kenny Stefania Marcotti Deandra Belo De Freitas Elena M Drudi Vivienne Leech Rachel E Bell Jennifer Easton Mar ía-Del-Carmen Díaz-de-la-Loza Roland Fleck Leanne Allison Christina Philippeos Angelika Manhart Tanya Shaw Brian M Stramer Source Type: research
En route towards a personalized medicine approach: Innovative therapeutic modalities for connective tissue disorders
Matrix Biol. 2023 Aug 30:S0945-053X(23)00092-6. doi: 10.1016/j.matbio.2023.08.005. Online ahead of print.ABSTRACTConnective tissue disorders can be caused by pathogenic variants (mutations) in genes encoding extracellular matrix (ECM) proteins. Such disorders typically manifest during development or postnatal growth and result in significant morbidity and mortality. The development of curative treatments for connective tissue disorders is hampered in part by the inability of many mature connective tissues to efficiently regenerate. To be most effective, therapeutic strategies designed to preserve or restore tissue function...
Source: Matrix Biology - September 1, 2023 Category: Molecular Biology Authors: Charlene Redhead Nandaraj Taye Dirk Hubmacher Source Type: research
