Francesco Ramirez
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View article: HYPOTHETICAL IN VIVO BEHAVIOR OF CARBAMAZEPINE TABLETS FROM IN VITRO RELEASE DATA OF USP APPARATUS II AND IV AND DISSOLUTION MEDIA OF PHYSIOLOGICAL RELEVANCE
HYPOTHETICAL IN VIVO BEHAVIOR OF CARBAMAZEPINE TABLETS FROM IN VITRO RELEASE DATA OF USP APPARATUS II AND IV AND DISSOLUTION MEDIA OF PHYSIOLOGICAL RELEVANCE Open
Objective: To estimate the hypothetical in vivo behavior of carbamazepine tablets (immediate-release, 200 mg) with dissolution data and a convolutional approach. Methods: USP apparatus II and IV and media at pH 1.2, 4.5, and 6.8 (all conta…
View article: Fibrillin-1 Deficiency Perturbs Aortic Cholinergic Relaxation and Adrenergic Contraction in a Mouse Model of Early Onset Progressively Severe Marfan Syndrome
Fibrillin-1 Deficiency Perturbs Aortic Cholinergic Relaxation and Adrenergic Contraction in a Mouse Model of Early Onset Progressively Severe Marfan Syndrome Open
Introduction: The pathogenic role of nitric oxide (NO) signaling during development of thoracic aortic aneurysm (TAA) in Marfan syndrome (MFS) is currently unclear. We characterized vasomotor function and its relationship to the activity o…
View article: Dissecting aortic aneurysm in Marfan syndrome is associated with losartan-sensitive transcriptomic modulation of aortic cells
Dissecting aortic aneurysm in Marfan syndrome is associated with losartan-sensitive transcriptomic modulation of aortic cells Open
To improve our limited understanding of the pathogenesis of thoracic aortic aneurysm (TAA) that leads to acute aortic dissection, single-cell RNA sequencing (scRNA-seq) was employed to profile disease-relevant transcriptomic changes of aor…
View article: Inhibition of HIPK2 Alleviates Thoracic Aortic Disease in Mice With Progressively Severe Marfan Syndrome
Inhibition of HIPK2 Alleviates Thoracic Aortic Disease in Mice With Progressively Severe Marfan Syndrome Open
Objective: Despite considerable research, the goal of finding nonsurgical remedies against thoracic aortic aneurysm and acute aortic dissection remains elusive. We sought to identify a novel aortic PK (protein kinase) that can be pharmacol…
View article: Nanoindentation of iPSC-Derived Aortic Smooth Muscle Cells Reveals a Biomechanical and Developmental Basis for Aneurysm Localization in Marfan Syndrome
Nanoindentation of iPSC-Derived Aortic Smooth Muscle Cells Reveals a Biomechanical and Developmental Basis for Aneurysm Localization in Marfan Syndrome Open
View article: Therapies for Thoracic Aortic Aneurysms and Acute Aortic Dissections
Therapies for Thoracic Aortic Aneurysms and Acute Aortic Dissections Open
Thoracic aortic aneurysms that progress to acute aortic dissections are often fatal. Thoracic aneurysms have been managed with treatment with β-adrenergic blocking agents (β-blockers) and routine surveillance imaging, followed by surgical …
View article: Cell Type–Specific Contributions of the Angiotensin II Type 1a Receptor to Aorta Homeostasis and Aneurysmal Disease—Brief Report
Cell Type–Specific Contributions of the Angiotensin II Type 1a Receptor to Aorta Homeostasis and Aneurysmal Disease—Brief Report Open
Objective— Two were the aims of this study: first, to translate whole-genome expression profiles into computational predictions of functional associations between signaling pathways that regulate aorta homeostasis and the activity of angio…
View article: Marfan syndrome; A connective tissue disease at the crossroads of mechanotransduction, TGFβ signaling and cell stemness
Marfan syndrome; A connective tissue disease at the crossroads of mechanotransduction, TGFβ signaling and cell stemness Open
View article: Therapeutics Targeting Drivers of Thoracic Aortic Aneurysms and Acute Aortic Dissections: Insights from Predisposing Genes and Mouse Models
Therapeutics Targeting Drivers of Thoracic Aortic Aneurysms and Acute Aortic Dissections: Insights from Predisposing Genes and Mouse Models Open
Thoracic aortic diseases, including aneurysms and dissections of the thoracic aorta, are a major cause of morbidity and mortality. Risk factors for thoracic aortic disease include increased hemodynamic forces on the ascending aorta, typica…
View article: Supplementary material from "Comparison of 10 murine models reveals a distinct biomechanical phenotype in thoracic aortic aneurysms"
Supplementary material from "Comparison of 10 murine models reveals a distinct biomechanical phenotype in thoracic aortic aneurysms" Open
Thoracic aortic aneurysms are life-threatening lesions that afflict young and old individuals alike. They frequently associate with genetic mutations and are characterized by reduced elastic fibre integrity, dysfunctional smooth muscle cel…
View article: Loss of elastic fiber integrity compromises common carotid artery function: Implications for vascular aging
Loss of elastic fiber integrity compromises common carotid artery function: Implications for vascular aging Open
Competent elastic fibers endow central arteries with the compliance and resilience that are fundamental to their primary mechanical function in vertebrates. That is, by enabling elastic energy to be stored in the arterial wall during systo…
View article: Genetic analysis of the contribution of LTBP-3 to thoracic aneurysm in Marfan syndrome
Genetic analysis of the contribution of LTBP-3 to thoracic aneurysm in Marfan syndrome Open
Significance Marfan syndrome (MFS) is a connective tissue disorder caused by mutations of fibrillin-1 (FBN1), the main component of extracellular matrix microfibrils. FBN1 mutations predispose to thoracic aortic aneurysm and rupture and ar…
View article: Abnormal Activation of BMP Signaling Causes Myopathy in Fbn2 Null Mice
Abnormal Activation of BMP Signaling Causes Myopathy in Fbn2 Null Mice Open
Fibrillins are large extracellular macromolecules that polymerize to form the backbone structure of connective tissue microfibrils. Mutations in the gene for fibrillin-1 cause the Marfan syndrome, while mutations in the gene for fibrillin-…
View article: Dimorphic Effects of Transforming Growth Factor-β Signaling During Aortic Aneurysm Progression in Mice Suggest a Combinatorial Therapy for Marfan Syndrome
Dimorphic Effects of Transforming Growth Factor-β Signaling During Aortic Aneurysm Progression in Mice Suggest a Combinatorial Therapy for Marfan Syndrome Open
Objective— Studies of mice with mild Marfan syndrome (MFS) have correlated the development of thoracic aortic aneurysm (TAA) with improper stimulation of noncanonical (Erk-mediated) TGFβ signaling by the angiotensin type I receptor (AT1r).…
View article: AFM Reveals Age-Dependent Micromechanical Degradation of Cardiopulmonary Tissues in a Mouse Model of Marfan Syndrome
AFM Reveals Age-Dependent Micromechanical Degradation of Cardiopulmonary Tissues in a Mouse Model of Marfan Syndrome Open