Robert P. Mecham

Identification of tropoelastin as a ligand for the 65-kD FK506-binding protein, FKBP65, in the secretory pathway.

Characterization of an in vitro model of elastic fiber assembly.

The microfibrillar proteins MAGP-1 and fibrillin-1 form a ternary complex with the chondroitin sulfate proteoglycan decorin.

Lysyl oxidase is required for vascular and diaphragmatic development in mice.

Hypertension and prolonged vasoconstrictor signaling in RGS2-deficient mice.

Domains in tropoelastin that mediate elastin deposition in vitro and in vivo.

A site on laminin alpha 5, AQARSAASKVKVSMKF, induces inflammatory cell production of matrix metalloproteinase-9 and chemotaxis.

Fibrillin-1 and -2 contain heparin-binding sites important for matrix deposition and that support cell attachment.

Dexamethasone induction of hypertension and diabetes is PPAR-alpha dependent in LDL receptor-null mice.

Developmental adaptation of the mouse cardiovascular system to elastin haploinsufficiency.

Identification of a major microfibril-associated glycoprotein-1-binding domain in fibrillin-2.

Neutrophil serine proteinases inactivate surfactant protein D by cleaving within a conserved subregion of the carbohydrate recognition domain.

Deposition of tropoelastin into the extracellular matrix requires a competent elastic fiber scaffold but not live cells.

Vascular extracellular matrix and aortic development.

TGF-beta-dependent pathogenesis of mitral valve prolapse in a mouse model of Marfan syndrome.

Altered vascular remodeling in fibulin-5-deficient mice reveals a role of fibulin-5 in smooth muscle cell proliferation and migration.

Effects of elastin haploinsufficiency on the mechanical behavior of mouse arteries.

A functional mutation in the terminal exon of elastin in severe, early-onset chronic obstructive pulmonary disease.

Tropoelastin interacts with cell-surface glycosaminoglycans via its COOH-terminal domain.

Elastic fiber formation: a dynamic view of extracellular matrix assembly using timer reporters.

Elastin fragments drive disease progression in a murine model of emphysema.

Specific sequence motifs direct the oxygenation and chlorination of tryptophan by myeloperoxidase.

Chlamydomonas agglutinin is a hydroxyproline-rich glycoprotein.

The stumbling block in lung repair of emphysema: elastic fiber assembly.

Fibulin-5 mutations: mechanisms of impaired elastic fiber formation in recessive cutis laxa.

Domains 16 and 17 of tropoelastin in elastic fibre formation.

Elastin protein levels are a vital modifier affecting normal lung development and susceptibility to emphysema.

Elastin-insufficient mice show normal cardiovascular remodeling in 2K1C hypertension despite higher baseline pressure and unique cardiovascular architecture.

Functional rescue of elastin insufficiency in mice by the human elastin gene: implications for mouse models of human disease.

The intracellular form of human MAGP1 elicits a complex and specific transcriptional response.

Fibulin-2 is dispensable for mouse development and elastic fiber formation.

Elastin haploinsufficiency induces alternative aging processes in the aorta.

Neutrophil elastase cleaves laminin-332 (laminin-5) generating peptides that are chemotactic for neutrophils.

New insights into elastic fiber assembly.

Mice lacking the extracellular matrix protein MAGP1 display delayed thrombotic occlusion following vessel injury.

Methods in elastic tissue biology: elastin isolation and purification.

Upregulation of elastase proteins results in aortic dilatation in mucopolysaccharidosis I mice.

Modification and functional inactivation of the tropoelastin carboxy-terminal domain in cross-linked elastin.

Deficiency in microfibril-associated glycoprotein-1 leads to complex phenotypes in multiple organ systems.

Elastin insufficiency predisposes to elevated pulmonary circulatory pressures through changes in elastic artery structure.

Essential role for fibrillin-2 in zebrafish notochord and vascular morphogenesis.

Fibrillins, fibulins, and matrix-associated glycoprotein modulate the kinetics and morphology of in vitro self-assembly of a recombinant elastin-like polypeptide.

Reduced vessel elasticity alters cardiovascular structure and function in newborn mice.

Effects of chronic treatment with a low dose of nicorandil on the function of the rat aorta during ageing.

Vascular extracellular matrix and arterial mechanics.

Discrete contributions of elastic fiber components to arterial development and mechanical compliance.

Microfibril-associated glycoprotein-1, an extracellular matrix regulator of bone remodeling.

Mechanisms of emphysema in autosomal dominant cutis laxa.

The matrix-binding domain of microfibril-associated glycoprotein-1 targets active connective tissue growth factor to a fibroblast-produced extracellular matrix.

Oxidative and nitrosative modifications of tropoelastin prevent elastic fiber assembly in vitro.

Evidence for heterozygous abnormalities of the elastin gene (ELN) affecting the quantity of vocal fold elastic fibers: a pilot study.

New insights into the pathogenesis of autosomal-dominant cutis laxa with report of five ELN mutations.

Oxidative modifications of the C-terminal domain of tropoelastin prevent cell binding.

Decreased aortic diameter and compliance precedes blood pressure increases in postnatal development of elastin-insufficient mice.

Characterization of erectile function in elastin haploinsufficicent mice.

Oophorectomy-induced bone loss is attenuated in MAGP1-deficient mice.

Pathogenesis of aortic dilatation in mucopolysaccharidosis VII mice may involve complement activation.

Genetic modifiers of cardiovascular phenotype caused by elastin haploinsufficiency act by extrinsic noncomplementation.

Inhibition of MicroRNA-29 Enhances Elastin Levels in Cells Haploinsufficient for Elastin and in Bioengineered Vessels.

Alternative splicing and tissue-specific elastin misassembly act as biological modifiers of human elastin gene frameshift mutations associated with dominant cutis laxa.

A fiber-based constitutive model predicts changes in amount and organization of matrix proteins with development and disease in the mouse aorta.

Paxillin contracts the osteoclast cytoskeleton.

Overview of extracellular matrix.

Development, composition, and structural arrangements of the ciliary zonule of the mouse.

Zap70 inhibits Syk-mediated osteoclast function.

Microfibril-associated glycoprotein 2 (MAGP2) loss of function has pleiotropic effects in vivo.

Hypotension due to Kir6.1 gain-of-function in vascular smooth muscle.

Ocular phenotype of Fbn2-null mice.

Nonselective assembly of fibrillin 1 and fibrillin 2 in the rodent ocular zonule and in cultured cells: implications for Marfan syndrome.

Altered reactivity of resistance vasculature contributes to hypertension in elastin insufficiency.

The extracellular matrix protein MAGP1 supports thermogenesis and protects against obesity and diabetes through regulation of TGF-β.

Fibroblast growth factor 2 is required for epithelial recovery, but not for pulmonary fibrosis, in response to bleomycin.

Adamtsl2 deletion results in bronchial fibrillin microfibril accumulation and bronchial epithelial dysplasia--a novel mouse model providing insights into geleophysic dysplasia.

The microfibril-associated glycoproteins (MAGPs) and the microfibrillar niche.

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.

Chronic antihypertensive treatment improves pulse pressure but not large artery mechanics in a mouse model of congenital vascular stiffness.

Characterization of metabolic health in mouse models of fibrillin-1 perturbation.

Albumin contributes to kidney disease progression in Alport syndrome.

Loss of function mutation in LOX causes thoracic aortic aneurysm and dissection in humans.

Marrow Adipose Tissue Expansion Coincides with Insulin Resistance in MAGP1-Deficient Mice.

Deficient Circumferential Growth Is the Primary Determinant of Aortic Obstruction Attributable to Partial Elastin Deficiency.

Inhibition of MAPK-Erk pathway in vivo attenuates aortic valve disease processes in Emilin1-deficient mouse model.

Fibulin-4 is essential for maintaining arterial wall integrity in conduit but not muscular arteries.

Downregulation of Lysyl Oxidase Protects Retinal Endothelial Cells From High Glucose-Induced Apoptosis.

FGF receptors control alveolar elastogenesis.

Measurement of elastin, collagen, and total protein levels in tissues.

Elastin purification and solubilization.

Elastin in lung development and disease pathogenesis.

Contribution of metabolic disease to bone fragility in MAGP1-deficient mice.

Microfibril-associated glycoproteins MAGP-1 and MAGP-2 in disease.

Requisite endothelial reactivation and effective siRNA nanoparticle targeting of Etv2/Er71 in tumor angiogenesis.

Spatial and temporal changes in extracellular elastin and laminin distribution during lung alveolar development.

Extracellular Determinants of Arterial Morphogenesis, Growth, and Homeostasis.

Cardiovascular consequences of KATP overactivity in Cantu syndrome.

Decreased lysyl oxidase level protects against development of retinal vascular lesions in diabetic retinopathy.

Intracellular retention of mutant lysyl oxidase leads to aortic dilation in response to increased hemodynamic stress.

Sodium-activated potassium channels moderate excitability in vascular smooth muscle.

Elastic fiber ultrastructure and assembly.

Identification of the growth factor-binding sequence in the extracellular matrix protein MAGP-1.

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.

A matricellular protein fibulin-4 is essential for the activation of lysyl oxidase.

RNA Vaccines for COVID-19: 5 Things Every Cardiologist Should Know.

Complex consequences of Cantu syndrome SUR2 variant R1154Q in genetically modified mice.

SVEP1 is a human coronary artery disease locus that promotes atherosclerosis.

Inhibition of NOX1 Mitigates Blood Pressure Increases in Elastin Insufficiency.

JNK2 Gene Silencing for Elastic Matrix Regenerative Repair.

Tissue-specific smooth muscle cell subtypes identified by transcriptional profiling.

Vascular Smooth Muscle Cell Subpopulations and Neointimal Formation in Mouse Models of Elastin Insufficiency.

Deletion of type VIII collagen reduces blood pressure, increases carotid artery functional distensibility and promotes elastin deposition.

Loss of Angiotensin II Type 2 Receptor Improves Blood Pressure in Elastin Insufficiency.