Robert P. Mecham

Department of Cell Biology & Physiology, Washington University School of Medicine

Affiliated withWashington University School of Medicine

Research Area

Biography

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JoVE Journal Publications

ArticleTotal : 1	Year
Publication title Cited by 3	2021

Other Publications

Article	Year
Identification of tropoelastin as a ligand for the 65-kD FK506-binding protein, FKBP65, in the secretory pathway. The Journal of cell biology\| PubMed ID: 9442105	1998
Characterization of an in vitro model of elastic fiber assembly. Molecular biology of the cell\| PubMed ID: 10564258	1999
The microfibrillar proteins MAGP-1 and fibrillin-1 form a ternary complex with the chondroitin sulfate proteoglycan decorin. Molecular biology of the cell\| PubMed ID: 10793130	2000
Lysyl oxidase is required for vascular and diaphragmatic development in mice. The Journal of biological chemistry\| PubMed ID: 12473682	2003
Hypertension and prolonged vasoconstrictor signaling in RGS2-deficient mice. The Journal of clinical investigation\| PubMed ID: 12588882	2003
Domains in tropoelastin that mediate elastin deposition in vitro and in vivo. The Journal of biological chemistry\| PubMed ID: 12626514	2003
A site on laminin alpha 5, AQARSAASKVKVSMKF, induces inflammatory cell production of matrix metalloproteinase-9 and chemotaxis. Journal of immunology (Baltimore, Md. : 1950)\| PubMed ID: 12817023	2003
Fibrillin-1 and -2 contain heparin-binding sites important for matrix deposition and that support cell attachment. The Biochemical journal\| PubMed ID: 12837131	2003
Dexamethasone induction of hypertension and diabetes is PPAR-alpha dependent in LDL receptor-null mice. Nature medicine\| PubMed ID: 12847522	2003
Developmental adaptation of the mouse cardiovascular system to elastin haploinsufficiency. The Journal of clinical investigation\| PubMed ID: 14597767	2003
Identification of a major microfibril-associated glycoprotein-1-binding domain in fibrillin-2. The Journal of biological chemistry\| PubMed ID: 15044481	2004
Neutrophil serine proteinases inactivate surfactant protein D by cleaving within a conserved subregion of the carbohydrate recognition domain. The Journal of biological chemistry\| PubMed ID: 15078883	2004
Deposition of tropoelastin into the extracellular matrix requires a competent elastic fiber scaffold but not live cells. Matrix biology : journal of the International Society for Matrix Biology\| PubMed ID: 15172035	2004
Vascular extracellular matrix and aortic development. Current topics in developmental biology\| PubMed ID: 15522742	2004
TGF-beta-dependent pathogenesis of mitral valve prolapse in a mouse model of Marfan syndrome. The Journal of clinical investigation\| PubMed ID: 15546004	2004
Altered vascular remodeling in fibulin-5-deficient mice reveals a role of fibulin-5 in smooth muscle cell proliferation and migration. Proceedings of the National Academy of Sciences of the United States of America\| PubMed ID: 15710889	2005
Effects of elastin haploinsufficiency on the mechanical behavior of mouse arteries. American journal of physiology. Heart and circulatory physiology\| PubMed ID: 15863465	2005
A functional mutation in the terminal exon of elastin in severe, early-onset chronic obstructive pulmonary disease. American journal of respiratory cell and molecular biology\| PubMed ID: 16081882	2005
Tropoelastin interacts with cell-surface glycosaminoglycans via its COOH-terminal domain. The Journal of biological chemistry\| PubMed ID: 16192266	2005
Elastic fiber formation: a dynamic view of extracellular matrix assembly using timer reporters. Journal of cellular physiology\| PubMed ID: 16261592	2006
Elastin fragments drive disease progression in a murine model of emphysema. The Journal of clinical investigation\| PubMed ID: 16470245	2006
Specific sequence motifs direct the oxygenation and chlorination of tryptophan by myeloperoxidase. Biochemistry\| PubMed ID: 16548523	2006
Chlamydomonas agglutinin is a hydroxyproline-rich glycoprotein. Proceedings of the National Academy of Sciences of the United States of America\| PubMed ID: 16593377	1983
The stumbling block in lung repair of emphysema: elastic fiber assembly. Proceedings of the American Thoracic Society\| PubMed ID: 16799087	2006
Fibulin-5 mutations: mechanisms of impaired elastic fiber formation in recessive cutis laxa. Human molecular genetics\| PubMed ID: 17035250	2006
Domains 16 and 17 of tropoelastin in elastic fibre formation. The Biochemical journal\| PubMed ID: 17037986	2007
Elastin protein levels are a vital modifier affecting normal lung development and susceptibility to emphysema. American journal of physiology. Lung cellular and molecular physiology\| PubMed ID: 17142349	2007
Elastin-insufficient mice show normal cardiovascular remodeling in 2K1C hypertension despite higher baseline pressure and unique cardiovascular architecture. American journal of physiology. Heart and circulatory physiology\| PubMed ID: 17400710	2007
Functional rescue of elastin insufficiency in mice by the human elastin gene: implications for mouse models of human disease. Circulation research\| PubMed ID: 17626896	2007
The intracellular form of human MAGP1 elicits a complex and specific transcriptional response. The international journal of biochemistry & cell biology\| PubMed ID: 17692555	2007
Fibulin-2 is dispensable for mouse development and elastic fiber formation. Molecular and cellular biology\| PubMed ID: 18070922	2008
Elastin haploinsufficiency induces alternative aging processes in the aorta. Rejuvenation research\| PubMed ID: 18173368	2008
Neutrophil elastase cleaves laminin-332 (laminin-5) generating peptides that are chemotactic for neutrophils. The Journal of biological chemistry\| PubMed ID: 18178964	2008
New insights into elastic fiber assembly. Birth defects research. Part C, Embryo today : reviews\| PubMed ID: 18228265	2007
Mice lacking the extracellular matrix protein MAGP1 display delayed thrombotic occlusion following vessel injury. Blood\| PubMed ID: 18281502	2008
Methods in elastic tissue biology: elastin isolation and purification. Methods (San Diego, Calif.)\| PubMed ID: 18442703	2008
Upregulation of elastase proteins results in aortic dilatation in mucopolysaccharidosis I mice. Molecular genetics and metabolism\| PubMed ID: 18479957	2008
Modification and functional inactivation of the tropoelastin carboxy-terminal domain in cross-linked elastin. Matrix biology : journal of the International Society for Matrix Biology\| PubMed ID: 18602002	2008
Deficiency in microfibril-associated glycoprotein-1 leads to complex phenotypes in multiple organ systems. The Journal of biological chemistry\| PubMed ID: 18625713	2008
Elastin insufficiency predisposes to elevated pulmonary circulatory pressures through changes in elastic artery structure. Journal of applied physiology (Bethesda, Md. : 1985)\| PubMed ID: 18772328	2008
Essential role for fibrillin-2 in zebrafish notochord and vascular morphogenesis. Developmental dynamics : an official publication of the American Association of Anatomists\| PubMed ID: 18816837	2008
Fibrillins, fibulins, and matrix-associated glycoprotein modulate the kinetics and morphology of in vitro self-assembly of a recombinant elastin-like polypeptide. Biochemistry\| PubMed ID: 18973305	2008
Reduced vessel elasticity alters cardiovascular structure and function in newborn mice. Circulation research\| PubMed ID: 19372465	2009
Effects of chronic treatment with a low dose of nicorandil on the function of the rat aorta during ageing. Clinical and experimental pharmacology & physiology\| PubMed ID: 19473347	2009
Vascular extracellular matrix and arterial mechanics. Physiological reviews\| PubMed ID: 19584318	2009
Discrete contributions of elastic fiber components to arterial development and mechanical compliance. Arteriosclerosis, thrombosis, and vascular biology\| PubMed ID: 19850904	2009
Microfibril-associated glycoprotein-1, an extracellular matrix regulator of bone remodeling. The Journal of biological chemistry\| PubMed ID: 20501659	2010
Mechanisms of emphysema in autosomal dominant cutis laxa. Matrix biology : journal of the International Society for Matrix Biology\| PubMed ID: 20600892	2010
The matrix-binding domain of microfibril-associated glycoprotein-1 targets active connective tissue growth factor to a fibroblast-produced extracellular matrix. Macromolecular bioscience\| PubMed ID: 20799254	2010
Oxidative and nitrosative modifications of tropoelastin prevent elastic fiber assembly in vitro. The Journal of biological chemistry\| PubMed ID: 20847053	2010
Evidence for heterozygous abnormalities of the elastin gene (ELN) affecting the quantity of vocal fold elastic fibers: a pilot study. Journal of voice : official journal of the Voice Foundation\| PubMed ID: 20926252	2011
New insights into the pathogenesis of autosomal-dominant cutis laxa with report of five ELN mutations. Human mutation\| PubMed ID: 21309044	2011
Oxidative modifications of the C-terminal domain of tropoelastin prevent cell binding. The Journal of biological chemistry\| PubMed ID: 21321118	2011
Decreased aortic diameter and compliance precedes blood pressure increases in postnatal development of elastin-insufficient mice. American journal of physiology. Heart and circulatory physiology\| PubMed ID: 21536846	2011
Characterization of erectile function in elastin haploinsufficicent mice. The journal of sexual medicine\| PubMed ID: 21883953	2011
Oophorectomy-induced bone loss is attenuated in MAGP1-deficient mice. Journal of cellular biochemistry\| PubMed ID: 21898536	2012
Pathogenesis of aortic dilatation in mucopolysaccharidosis VII mice may involve complement activation. Molecular genetics and metabolism\| PubMed ID: 21944884	2011
Genetic modifiers of cardiovascular phenotype caused by elastin haploinsufficiency act by extrinsic noncomplementation. The Journal of biological chemistry\| PubMed ID: 22049077	2011
Inhibition of MicroRNA-29 Enhances Elastin Levels in Cells Haploinsufficient for Elastin and in Bioengineered Vessels. Arteriosclerosis, thrombosis, and vascular biology\| PubMed ID: 22095981	2011
Alternative splicing and tissue-specific elastin misassembly act as biological modifiers of human elastin gene frameshift mutations associated with dominant cutis laxa. The Journal of biological chemistry\| PubMed ID: 22573328	2012
A fiber-based constitutive model predicts changes in amount and organization of matrix proteins with development and disease in the mouse aorta. Biomechanics and modeling in mechanobiology\| PubMed ID: 22790326	2013
Paxillin contracts the osteoclast cytoskeleton. Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research\| PubMed ID: 22807029	2012
Overview of extracellular matrix. Current protocols in cell biology\| PubMed ID: 23208544	2012
Development, composition, and structural arrangements of the ciliary zonule of the mouse. Investigative ophthalmology & visual science\| PubMed ID: 23493297	2013
Zap70 inhibits Syk-mediated osteoclast function. Journal of cellular biochemistry\| PubMed ID: 23494777	2013
Microfibril-associated glycoprotein 2 (MAGP2) loss of function has pleiotropic effects in vivo. The Journal of biological chemistry\| PubMed ID: 23963447	2013
Hypotension due to Kir6.1 gain-of-function in vascular smooth muscle. Journal of the American Heart Association\| PubMed ID: 23974906	2013
Ocular phenotype of Fbn2-null mice. Investigative ophthalmology & visual science\| PubMed ID: 24130178	2013
Nonselective assembly of fibrillin 1 and fibrillin 2 in the rodent ocular zonule and in cultured cells: implications for Marfan syndrome. Investigative ophthalmology & visual science\| PubMed ID: 24265020	2013
Altered reactivity of resistance vasculature contributes to hypertension in elastin insufficiency. American journal of physiology. Heart and circulatory physiology\| PubMed ID: 24414067	2014
The extracellular matrix protein MAGP1 supports thermogenesis and protects against obesity and diabetes through regulation of TGF-β. Diabetes\| PubMed ID: 24458361	2014
Fibroblast growth factor 2 is required for epithelial recovery, but not for pulmonary fibrosis, in response to bleomycin. American journal of respiratory cell and molecular biology\| PubMed ID: 24988442	2015
Adamtsl2 deletion results in bronchial fibrillin microfibril accumulation and bronchial epithelial dysplasia--a novel mouse model providing insights into geleophysic dysplasia. Disease models & mechanisms\| PubMed ID: 25762570	2015
The microfibril-associated glycoproteins (MAGPs) and the microfibrillar niche. Matrix biology : journal of the International Society for Matrix Biology\| PubMed ID: 25963142	2015
Fibulin-4 E57K Knock-in Mice Recapitulate Cutaneous, Vascular and Skeletal Defects of Recessive Cutis Laxa 1B with both Elastic Fiber and Collagen Fibril Abnormalities. The Journal of biological chemistry\| PubMed ID: 26178373	2015
Chronic antihypertensive treatment improves pulse pressure but not large artery mechanics in a mouse model of congenital vascular stiffness. American journal of physiology. Heart and circulatory physiology\| PubMed ID: 26232234	2015
Characterization of metabolic health in mouse models of fibrillin-1 perturbation. Matrix biology : journal of the International Society for Matrix Biology\| PubMed ID: 26902431	2016
Albumin contributes to kidney disease progression in Alport syndrome. American journal of physiology. Renal physiology\| PubMed ID: 27147675	2016
Loss of function mutation in LOX causes thoracic aortic aneurysm and dissection in humans. Proceedings of the National Academy of Sciences of the United States of America\| PubMed ID: 27432961	2016
Marrow Adipose Tissue Expansion Coincides with Insulin Resistance in MAGP1-Deficient Mice. Frontiers in endocrinology\| PubMed ID: 27445989	2016
Deficient Circumferential Growth Is the Primary Determinant of Aortic Obstruction Attributable to Partial Elastin Deficiency. Arteriosclerosis, thrombosis, and vascular biology\| PubMed ID: 28254817	2017
Inhibition of MAPK-Erk pathway in vivo attenuates aortic valve disease processes in Emilin1-deficient mouse model. Physiological reports\| PubMed ID: 28270590	2017
Fibulin-4 is essential for maintaining arterial wall integrity in conduit but not muscular arteries. Science advances\| PubMed ID: 28508064	2017
Downregulation of Lysyl Oxidase Protects Retinal Endothelial Cells From High Glucose-Induced Apoptosis. Investigative ophthalmology & visual science\| PubMed ID: 28538980	2017
FGF receptors control alveolar elastogenesis. Development (Cambridge, England)\| PubMed ID: 29122839	2017
Measurement of elastin, collagen, and total protein levels in tissues. Methods in cell biology\| PubMed ID: 29310774	2018
Elastin purification and solubilization. Methods in cell biology\| PubMed ID: 29310779	2018
Elastin in lung development and disease pathogenesis. Matrix biology : journal of the International Society for Matrix Biology\| PubMed ID: 29331337	2018
Contribution of metabolic disease to bone fragility in MAGP1-deficient mice. Matrix biology : journal of the International Society for Matrix Biology\| PubMed ID: 29519758	2018
Microfibril-associated glycoproteins MAGP-1 and MAGP-2 in disease. Matrix biology : journal of the International Society for Matrix Biology\| PubMed ID: 29524629	2018
Requisite endothelial reactivation and effective siRNA nanoparticle targeting of Etv2/Er71 in tumor angiogenesis. JCI insight\| PubMed ID: 29669933	2018
Spatial and temporal changes in extracellular elastin and laminin distribution during lung alveolar development. Scientific reports\| PubMed ID: 29844468	2018
Extracellular Determinants of Arterial Morphogenesis, Growth, and Homeostasis. Current topics in developmental biology\| PubMed ID: 29853177	2018
Cardiovascular consequences of KATP overactivity in Cantu syndrome. JCI insight\| PubMed ID: 30089727	2018
Decreased lysyl oxidase level protects against development of retinal vascular lesions in diabetic retinopathy. Experimental eye research\| PubMed ID: 31022398	2019
Intracellular retention of mutant lysyl oxidase leads to aortic dilation in response to increased hemodynamic stress. JCI insight\| PubMed ID: 31211696	2019
Sodium-activated potassium channels moderate excitability in vascular smooth muscle. The Journal of physiology\| PubMed ID: 31444905	2019
Elastic fiber ultrastructure and assembly. Matrix biology : journal of the International Society for Matrix Biology\| PubMed ID: 31669522	2019
Identification of the growth factor-binding sequence in the extracellular matrix protein MAGP-1. The Journal of biological chemistry\| PubMed ID: 31988245	2020
Whole exome sequencing in patients with Williams-Beuren syndrome followed by disease modeling in mice points to four novel pathways that may modify stenosis risk. Human molecular genetics\| PubMed ID: 32412588	2020
A matricellular protein fibulin-4 is essential for the activation of lysyl oxidase. Science advances\| PubMed ID: 33239290	2020
RNA Vaccines for COVID-19: 5 Things Every Cardiologist Should Know. JACC. Basic to translational science\| PubMed ID: 33251385	2020
Complex consequences of Cantu syndrome SUR2 variant R1154Q in genetically modified mice. JCI insight\| PubMed ID: 33529173	2021
SVEP1 is a human coronary artery disease locus that promotes atherosclerosis. Science translational medicine\| PubMed ID: 33762433	2021
Inhibition of NOX1 Mitigates Blood Pressure Increases in Elastin Insufficiency. Function (Oxford, England)\| PubMed ID: 34223172	2021
JNK2 Gene Silencing for Elastic Matrix Regenerative Repair. Tissue engineering. Part A\| PubMed ID: 34409851	2021
Tissue-specific smooth muscle cell subtypes identified by transcriptional profiling. The international journal of biochemistry & cell biology\| PubMed ID: 34411694	2021
Vascular Smooth Muscle Cell Subpopulations and Neointimal Formation in Mouse Models of Elastin Insufficiency. Arteriosclerosis, thrombosis, and vascular biology\| PubMed ID: 34587758	2021
Deletion of type VIII collagen reduces blood pressure, increases carotid artery functional distensibility and promotes elastin deposition. Matrix biology plus\| PubMed ID: 34693248	2021
Loss of Angiotensin II Type 2 Receptor Improves Blood Pressure in Elastin Insufficiency. Frontiers in cardiovascular medicine\| PubMed ID: 34790711	2021