Enhanced Optic Nerve Expansion and Altered Ultrastructure of Elastic Fibers Induced by Lysyl Oxidase Inhibition in a Mouse Model of Marfan Syndrome
Am J Pathol. 2024 Mar 26:S0002-9440(24)00115-9. doi: 10.1016/j.ajpath.2024.03.002. Online ahead of print.ABSTRACTTwo major constituents of exfoliation material (XFM), fibrillin- 1 and lysyl oxidase-like 1 (encoded by FBN1 and LOXL1) are implicated in exfoliation glaucoma (XFG), yet their individual contributions to ocular phenotype are minor. To test the hypothesis that a combination of FBN1 mutation and LOXL1 deficiency exacerbates ocular phenotypes, pan-LOX inhibitor, β-aminopropionitrile (BAPN) was used to treat adult wild-type (wt) and Fbn1C1041G/+ mice for 8 weeks and their eyes were examined. Although intraocular pr...
Source: Am J Pathol - March 28, 2024 Category: Pathology Authors: Hang-Jing Wu Evan Krystofiak John Kuchtey Rachel W Kuchtey Source Type: research
Enhanced Optic Nerve Expansion and Altered Ultrastructure of Elastic Fibers Induced by Lysyl Oxidase Inhibition in a Mouse Model of Marfan Syndrome
Am J Pathol. 2024 Mar 26:S0002-9440(24)00115-9. doi: 10.1016/j.ajpath.2024.03.002. Online ahead of print.ABSTRACTTwo major constituents of exfoliation material, fibrillin-1 and lysyl oxidase-like 1 (encoded by FBN1 and LOXL1), are implicated in exfoliation glaucoma, yet their individual contributions to ocular phenotype are minor. To test the hypothesis that a combination of FBN1 mutation and LOXL1 deficiency exacerbates ocular phenotypes, the pan-LOX inhibitor β-aminopropionitrile (BAPN) was used to treat adult wild-type (WT) and Fbn1C1041G/+ mice for 8 weeks and their eyes were examined. Although intraocular pressure di...
Source: The American Journal of Pathology - March 28, 2024 Category: Pathology Authors: Hang-Jing Wu Evan Krystofiak John Kuchtey Rachel W Kuchtey Source Type: research
Enhanced Optic Nerve Expansion and Altered Ultrastructure of Elastic Fibers Induced by Lysyl Oxidase Inhibition in a Mouse Model of Marfan Syndrome
Am J Pathol. 2024 Mar 26:S0002-9440(24)00115-9. doi: 10.1016/j.ajpath.2024.03.002. Online ahead of print.ABSTRACTTwo major constituents of exfoliation material, fibrillin-1 and lysyl oxidase-like 1 (encoded by FBN1 and LOXL1), are implicated in exfoliation glaucoma, yet their individual contributions to ocular phenotype are minor. To test the hypothesis that a combination of FBN1 mutation and LOXL1 deficiency exacerbates ocular phenotypes, the pan-LOX inhibitor β-aminopropionitrile (BAPN) was used to treat adult wild-type (WT) and Fbn1C1041G/+ mice for 8 weeks and their eyes were examined. Although intraocular pressure di...
Source: Am J Pathol - March 28, 2024 Category: Pathology Authors: Hang-Jing Wu Evan Krystofiak John Kuchtey Rachel W Kuchtey Source Type: research
Enhanced Optic Nerve Expansion and Altered Ultrastructure of Elastic Fibers Induced by Lysyl Oxidase Inhibition in a Mouse Model of Marfan Syndrome
Am J Pathol. 2024 Mar 26:S0002-9440(24)00115-9. doi: 10.1016/j.ajpath.2024.03.002. Online ahead of print.ABSTRACTTwo major constituents of exfoliation material, fibrillin-1 and lysyl oxidase-like 1 (encoded by FBN1 and LOXL1), are implicated in exfoliation glaucoma, yet their individual contributions to ocular phenotype are minor. To test the hypothesis that a combination of FBN1 mutation and LOXL1 deficiency exacerbates ocular phenotypes, the pan-LOX inhibitor β-aminopropionitrile (BAPN) was used to treat adult wild-type (WT) and Fbn1C1041G/+ mice for 8 weeks and their eyes were examined. Although intraocular pressure di...
Source: The American Journal of Pathology - March 28, 2024 Category: Pathology Authors: Hang-Jing Wu Evan Krystofiak John Kuchtey Rachel W Kuchtey Source Type: research
Enhanced Optic Nerve Expansion and Altered Ultrastructure of Elastic Fibers Induced by Lysyl Oxidase Inhibition in a Mouse Model of Marfan Syndrome
Am J Pathol. 2024 Mar 26:S0002-9440(24)00115-9. doi: 10.1016/j.ajpath.2024.03.002. Online ahead of print.ABSTRACTTwo major constituents of exfoliation material, fibrillin-1 and lysyl oxidase-like 1 (encoded by FBN1 and LOXL1), are implicated in exfoliation glaucoma, yet their individual contributions to ocular phenotype are minor. To test the hypothesis that a combination of FBN1 mutation and LOXL1 deficiency exacerbates ocular phenotypes, the pan-LOX inhibitor β-aminopropionitrile (BAPN) was used to treat adult wild-type (WT) and Fbn1C1041G/+ mice for 8 weeks and their eyes were examined. Although intraocular pressure di...
Source: Am J Pathol - March 28, 2024 Category: Pathology Authors: Hang-Jing Wu Evan Krystofiak John Kuchtey Rachel W Kuchtey Source Type: research
Enhanced Optic Nerve Expansion and Altered Ultrastructure of Elastic Fibers Induced by Lysyl Oxidase Inhibition in a Mouse Model of Marfan Syndrome
Two major constituents of exfoliation material (XFM), fibrillin- 1 and lysyl oxidase-like 1 (encoded by FBN1 and LOXL1) are implicated in exfoliation glaucoma (XFG), yet their individual contributions to ocular phenotype are minor. To test the hypothesis that a combination of FBN1 mutation and LOXL1 deficiency exacerbates ocular phenotypes, pan-LOX inhibitor, β-aminopropionitrile (BAPN) was used to treat adult wild-type (wt) and Fbn1C1041G/+ mice for 8 weeks and their eyes were examined. Although intraocular pressure was not changed and XFM not detected in the eyes, BAPN treatment worsened optic nerve and axon expansion i...
Source: American Journal of Pathology - March 26, 2024 Category: Pathology Authors: Hang-Jing Wu, Evan Krystofiak, John Kuchtey, Rachel W. Kuchtey Tags: Regular Article Source Type: research
Successful endovascular treatment of aortic arch aneurysm in a pediatric patient
CONCLUSION: The endovascular management of large vessel aneurysms using stents and microcatheters with embolization of the aneurysmal sac is a novel management option that achieves successful results.PMID:38507313 | DOI:10.24875/ACM.22000272 (Source: Archivos de Cardiologia de Mexico)
Source: Archivos de Cardiologia de Mexico - March 20, 2024 Category: Cardiology Authors: Isabel C S ánchez-Escobar Silvia J Galvis-Blanco Luis H D íaz-Medina Rafael Correa-Vel ásquez Rafael Lince-Varela Source Type: research
Successful endovascular treatment of aortic arch aneurysm in a pediatric patient
CONCLUSION: The endovascular management of large vessel aneurysms using stents and microcatheters with embolization of the aneurysmal sac is a novel management option that achieves successful results.PMID:38507313 | DOI:10.24875/ACM.22000272 (Source: Archivos de Cardiologia de Mexico)
Source: Archivos de Cardiologia de Mexico - March 20, 2024 Category: Cardiology Authors: Isabel C S ánchez-Escobar Silvia J Galvis-Blanco Luis H D íaz-Medina Rafael Correa-Vel ásquez Rafael Lince-Varela Source Type: research
Successful endovascular treatment of aortic arch aneurysm in a pediatric patient
CONCLUSION: The endovascular management of large vessel aneurysms using stents and microcatheters with embolization of the aneurysmal sac is a novel management option that achieves successful results.PMID:38507313 | DOI:10.24875/ACM.22000272 (Source: Archivos de Cardiologia de Mexico)
Source: Archivos de Cardiologia de Mexico - March 20, 2024 Category: Cardiology Authors: Isabel C S ánchez-Escobar Silvia J Galvis-Blanco Luis H D íaz-Medina Rafael Correa-Vel ásquez Rafael Lince-Varela Source Type: research
Successful endovascular treatment of aortic arch aneurysm in a pediatric patient
CONCLUSION: The endovascular management of large vessel aneurysms using stents and microcatheters with embolization of the aneurysmal sac is a novel management option that achieves successful results.PMID:38507313 | DOI:10.24875/ACM.22000272 (Source: Archivos de Cardiologia de Mexico)
Source: Archivos de Cardiologia de Mexico - March 20, 2024 Category: Cardiology Authors: Isabel C S ánchez-Escobar Silvia J Galvis-Blanco Luis H D íaz-Medina Rafael Correa-Vel ásquez Rafael Lince-Varela Source Type: research
Successful endovascular treatment of aortic arch aneurysm in a pediatric patient
CONCLUSION: The endovascular management of large vessel aneurysms using stents and microcatheters with embolization of the aneurysmal sac is a novel management option that achieves successful results.PMID:38507313 | DOI:10.24875/ACM.22000272 (Source: Archivos de Cardiologia de Mexico)
Source: Archivos de Cardiologia de Mexico - March 20, 2024 Category: Cardiology Authors: Isabel C S ánchez-Escobar Silvia J Galvis-Blanco Luis H D íaz-Medina Rafael Correa-Vel ásquez Rafael Lince-Varela Source Type: research
Corneal biomechanics and their association with severity of lens dislocation in Marfan syndrome
ConclusionMarfan patients were detected to have increased corneal stiffness compared with normal subjects. Corneal biomechanical parameters were significantly associated with the severity of lens dislocation in Marfan patients. (Source: International Ophthalmology)
Source: International Ophthalmology - March 19, 2024 Category: Opthalmology Source Type: research
The Impact of Pregnancy in Patients with Thoracic Aortic Disease: Epidemiology, Risk Assessment, and Management Considerations
Methodist Debakey Cardiovasc J. 2024 Mar 14;20(2):51-58. doi: 10.14797/mdcvj.1371. eCollection 2024.ABSTRACTThoracic aortic disease (TAD) poses substantial risks during pregnancy, particularly for women with genetic conditions such as Marfan syndrome, Loeys-Dietz syndrome, and vascular Ehlers-Danlos syndrome. This review examines the epidemiology, risk assessment, and management of TAD in pregnancy. Preconception counseling is vital considering the hereditary nature of TAD and potential pregnancy-related complications. Genetic testing and imaging surveillance aid in risk assessment. Medical management, including beta-block...
Source: Methodist DeBakey Cardiovascular Journal - March 18, 2024 Category: Cardiology Authors: Valeria E Duarte Jessica N Richardson Michael N Singh Source Type: research
The Impact of Pregnancy in Patients with Thoracic Aortic Disease: Epidemiology, Risk Assessment, and Management Considerations
Methodist Debakey Cardiovasc J. 2024 Mar 14;20(2):51-58. doi: 10.14797/mdcvj.1371. eCollection 2024.ABSTRACTThoracic aortic disease (TAD) poses substantial risks during pregnancy, particularly for women with genetic conditions such as Marfan syndrome, Loeys-Dietz syndrome, and vascular Ehlers-Danlos syndrome. This review examines the epidemiology, risk assessment, and management of TAD in pregnancy. Preconception counseling is vital considering the hereditary nature of TAD and potential pregnancy-related complications. Genetic testing and imaging surveillance aid in risk assessment. Medical management, including beta-block...
Source: Methodist DeBakey Cardiovascular Journal - March 18, 2024 Category: Cardiology Authors: Valeria E Duarte Jessica N Richardson Michael N Singh Source Type: research
The Impact of Pregnancy in Patients with Thoracic Aortic Disease: Epidemiology, Risk Assessment, and Management Considerations
Methodist Debakey Cardiovasc J. 2024 Mar 14;20(2):51-58. doi: 10.14797/mdcvj.1371. eCollection 2024.ABSTRACTThoracic aortic disease (TAD) poses substantial risks during pregnancy, particularly for women with genetic conditions such as Marfan syndrome, Loeys-Dietz syndrome, and vascular Ehlers-Danlos syndrome. This review examines the epidemiology, risk assessment, and management of TAD in pregnancy. Preconception counseling is vital considering the hereditary nature of TAD and potential pregnancy-related complications. Genetic testing and imaging surveillance aid in risk assessment. Medical management, including beta-block...
Source: Methodist DeBakey Cardiovascular Journal - March 18, 2024 Category: Cardiology Authors: Valeria E Duarte Jessica N Richardson Michael N Singh Source Type: research
