Published in Mol Genet Metab on August 24, 2011
Enhancing astrocytic lysosome biogenesis facilitates Aβ clearance and attenuates amyloid plaque pathogenesis. J Neurosci (2014) 1.00
Effect of neonatal gene therapy on lumbar spine disease in mucopolysaccharidosis VII dogs. Mol Genet Metab (2012) 0.92
Carotid intima-media thickness is increased in patients with treated mucopolysaccharidosis types I and II, and correlates with arterial stiffness. Mol Genet Metab (2013) 0.87
Neonatal gene therapy with a gamma retroviral vector in mucopolysaccharidosis VI cats. Mol Ther (2012) 0.85
Pathogenesis of lumbar spine disease in mucopolysaccharidosis VII. Mol Genet Metab (2012) 0.82
The effect of neonatal gene therapy on skeletal manifestations in mucopolysaccharidosis VII dogs after a decade. Mol Genet Metab (2013) 0.81
Immune-Mediated Inflammation May Contribute to the Pathogenesis of Cardiovascular Disease in Mucopolysaccharidosis Type I. PLoS One (2016) 0.80
Retroviral-vector-mediated gene therapy to mucopolysaccharidosis I mice improves sensorimotor impairments and other behavioral deficits. J Inherit Metab Dis (2012) 0.78
Characterization of joint disease in mucopolysaccharidosis type I mice. Int J Exp Pathol (2013) 0.78
Pathogenesis of mitral valve disease in mucopolysaccharidosis VII dogs. Mol Genet Metab (2013) 0.77
Encapsulated Whole Bone Marrow Cells Improve Survival in Wistar Rats after 90% Partial Hepatectomy. Stem Cells Int (2015) 0.76
Effects of enzyme replacement therapy started late in a murine model of mucopolysaccharidosis type I. PLoS One (2015) 0.75
The effect of Tlr4 and/or C3 deficiency and of neonatal gene therapy on skeletal disease in mucopolysaccharidosis VII mice. Mol Genet Metab (2014) 0.75
Losartan improves aortic dilatation and cardiovascular disease in mucopolysaccharidosis I. J Inherit Metab Dis (2017) 0.75
Angiotensin II type 2 receptor signaling attenuates aortic aneurysm in mice through ERK antagonism. Science (2011) 3.18
Activated microglia in cortex of mouse models of mucopolysaccharidoses I and IIIB. Proc Natl Acad Sci U S A (2003) 2.96
Metalloelastase is required for macrophage-mediated proteolysis and matrix invasion in mice. Proc Natl Acad Sci U S A (1996) 2.93
Deficiency of cathepsin S reduces atherosclerosis in LDL receptor-deficient mice. J Clin Invest (2003) 2.48
A follow-up study of MPS I patients treated with laronidase enzyme replacement therapy for 6 years. Mol Genet Metab (2006) 2.27
Effects of elastin haploinsufficiency on the mechanical behavior of mouse arteries. Am J Physiol Heart Circ Physiol (2005) 2.06
Cross-regulation of signaling by ITAM-associated receptors. Nat Immunol (2009) 2.06
Crosstalk pathways between Toll-like receptors and the complement system. Trends Immunol (2010) 1.83
Elastin. Adv Protein Chem (2005) 1.82
Pathophysiology of abdominal aortic aneurysms: insights from the elastase-induced model in mice with different genetic backgrounds. Ann N Y Acad Sci (2006) 1.80
MMPs as therapeutic targets--still a viable option? Semin Cell Dev Biol (2007) 1.78
Mechanism of glycosaminoglycan-mediated bone and joint disease: implications for the mucopolysaccharidoses and other connective tissue diseases. Am J Pathol (2007) 1.67
Impaired elastogenesis in Hurler disease: dermatan sulfate accumulation linked to deficiency in elastin-binding protein and elastic fiber assembly. Am J Pathol (2000) 1.66
Increased life span and correction of metabolic defects in murine mucopolysaccharidosis type VII after syngeneic bone marrow transplantation. Blood (1991) 1.61
Liver-directed neonatal gene therapy prevents cardiac, bone, ear, and eye disease in mucopolysaccharidosis I mice. Mol Ther (2005) 1.58
Essential role of mannose-binding lectin-associated serine protease-1 in activation of the complement factor D. J Exp Med (2009) 1.51
Deficiency of the cysteine protease cathepsin S impairs microvessel growth. Circ Res (2003) 1.51
Therapeutic neonatal hepatic gene therapy in mucopolysaccharidosis VII dogs. Proc Natl Acad Sci U S A (2002) 1.50
Elastinolytic activity of human cathepsin L. Biochem J (1986) 1.48
Regulation of collagenase activities of human cathepsins by glycosaminoglycans. J Biol Chem (2003) 1.46
Upregulation of elastase proteins results in aortic dilatation in mucopolysaccharidosis I mice. Mol Genet Metab (2008) 1.45
Correction of clinical manifestations of canine mucopolysaccharidosis I with neonatal retroviral vector gene therapy. Mol Ther (2007) 1.42
Cardiac findings after enzyme replacement therapy for mucopolysaccharidosis type I. Am J Cardiol (2006) 1.41
Involvement of the Toll-like receptor 4 pathway and use of TNF-alpha antagonists for treatment of the mucopolysaccharidoses. Proc Natl Acad Sci U S A (2009) 1.37
MMP-12 has a role in abdominal aortic aneurysms in mice. Surgery (2005) 1.35
Variation in the phenotypic expression of beta-glucuronidase deficiency. J Pediatr (1975) 1.33
Cardiac manifestations in the mouse model of mucopolysaccharidosis I. Mol Genet Metab (2005) 1.31
Early neurodegeneration progresses independently of microglial activation by heparan sulfate in the brain of mucopolysaccharidosis IIIB mice. PLoS One (2008) 1.30
Gene therapy for mucopolysaccharidosis. Expert Opin Biol Ther (2007) 1.28
Upregulation of elastase activity in aorta in mucopolysaccharidosis I and VII dogs may be due to increased cytokine expression. Mol Genet Metab (2009) 1.25
Replacement therapy in Mucopolysaccharidosis type VI: advantages of early onset of therapy. Mol Genet Metab (2003) 1.23
Monitoring compartment-specific substrate cleavage by cathepsins B, K, L, and S at physiological pH and redox conditions. BMC Biochem (2009) 1.21
Gene therapy ameliorates cardiovascular disease in dogs with mucopolysaccharidosis VII. Circulation (2004) 1.20
Usefulness of bone marrow transplantation in the Hurler syndrome. Am J Cardiol (2003) 1.17
The heart in the Hurler syndrome: gross, histologic and ultrastructural observations in five necropsy cases. Am J Cardiol (1976) 1.15
Interaction between human cathepsins K, L, and S and elastins: mechanism of elastinolysis and inhibition by macromolecular inhibitors. J Biol Chem (2007) 1.14
Complement-dependent neutrophil recruitment is critical for the development of elastase-induced abdominal aortic aneurysm. Circulation (2009) 1.13
Cardiac functional and histopathologic findings in humans and mice with mucopolysaccharidosis type I: implications for assessment of therapeutic interventions in hurler syndrome. Pediatr Res (2005) 1.12
The endogenous vascular elastase that governs development and progression of monocrotaline-induced pulmonary hypertension in rats is a novel enzyme related to the serine proteinase adipsin. J Clin Invest (1994) 1.09
Age-related changes in amounts and concentrations of collagen and elastin in normotensive human thoracic aorta. Clin Chim Acta (1996) 1.07
Evaluation of pathological manifestations of disease in mucopolysaccharidosis VII mice after neonatal hepatic gene therapy. Mol Ther (2002) 1.07
Recombinant human procathepsin S is capable of autocatalytic processing at neutral pH in the presence of glycosaminoglycans. FEBS Lett (2005) 1.00
The mild form of mucopolysaccharidosis type I (Scheie syndrome) is associated with increased ascending aortic stiffness. Heart Vessels (2008) 0.98
A self-inactivating gamma-retroviral vector reduces manifestations of mucopolysaccharidosis I in mice. Mol Ther (2009) 0.97
Effects of bone marrow transplantation on the cardiovascular abnormalities in canine mucopolysaccharidosis VII. Bone Marrow Transplant (2000) 0.96
Clinical characterization of cardiovascular abnormalities associated with feline mucopolysaccharidosis I and VI. J Inherit Metab Dis (2008) 0.95
Bone marrow transplantation for feline mucopolysaccharidosis I. Mol Genet Metab (2007) 0.95
Essential role of complement mannose-binding lectin-associated serine proteases-1/3 in the murine collagen antibody-induced model of inflammatory arthritis. J Immunol (2010) 0.94
Cardiac abnormalities in adults with the attenuated form of mucopolysaccharidosis type I. J Inherit Metab Dis (2007) 0.92
Activation of cathepsin B, secreted by a colorectal cancer cell line requires low pH and is mediated by cathepsin D. Int J Cancer (1996) 0.91
Bone marrow transplantation in newborn rats with mucopolysaccharidosis type VI: biochemical, pathological, and clinical findings. Transplantation (1997) 0.87
Cardiovascular changes after bone marrow transplantation in dogs with mucopolysaccharidosis I. Am J Vet Res (1990) 0.86
Metabolic adaptations to interrupted glycosaminoglycan recycling. J Biol Chem (2009) 0.83
TGF-beta-dependent pathogenesis of mitral valve prolapse in a mouse model of Marfan syndrome. J Clin Invest (2004) 4.85
Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome. Blood (2006) 4.51
Human T regulatory cells can use the perforin pathway to cause autologous target cell death. Immunity (2004) 4.11
Elastin fragments drive disease progression in a murine model of emphysema. J Clin Invest (2006) 3.11
Mutations in complement C3 predispose to development of atypical hemolytic uremic syndrome. Blood (2008) 3.10
Vascular extracellular matrix and arterial mechanics. Physiol Rev (2009) 3.05
Genetic and functional analyses of membrane cofactor protein (CD46) mutations in atypical hemolytic uremic syndrome. J Am Soc Nephrol (2006) 2.88
Activation of human CD4+ cells with CD3 and CD46 induces a T-regulatory cell 1 phenotype. Nature (2003) 2.83
Mutations in human complement regulator, membrane cofactor protein (CD46), predispose to development of familial hemolytic uremic syndrome. Proc Natl Acad Sci U S A (2003) 2.79
Hypertension and prolonged vasoconstrictor signaling in RGS2-deficient mice. J Clin Invest (2003) 2.68
Differential expression of granzymes A and B in human cytotoxic lymphocyte subsets and T regulatory cells. Blood (2004) 2.58
Adenovirus type 11 uses CD46 as a cellular receptor. J Virol (2003) 2.52
Virulence differences between monkeypox virus isolates from West Africa and the Congo basin. Virology (2005) 2.36
C-terminal truncations in human 3'-5' DNA exonuclease TREX1 cause autosomal dominant retinal vasculopathy with cerebral leukodystrophy. Nat Genet (2007) 2.35
T-cell regulation: with complements from innate immunity. Nat Rev Immunol (2006) 2.27
New insights into elastic fiber assembly. Birth Defects Res C Embryo Today (2007) 2.20
Immunology of age-related macular degeneration. Nat Rev Immunol (2013) 2.14
Effects of elastin haploinsufficiency on the mechanical behavior of mouse arteries. Am J Physiol Heart Circ Physiol (2005) 2.06
Developmental adaptation of the mouse cardiovascular system to elastin haploinsufficiency. J Clin Invest (2003) 2.05
Properdin can initiate complement activation by binding specific target surfaces and providing a platform for de novo convertase assembly. J Immunol (2007) 2.00
Secreted NS1 of dengue virus attaches to the surface of cells via interactions with heparan sulfate and chondroitin sulfate E. PLoS Pathog (2007) 1.97
West Nile virus nonstructural protein NS1 inhibits complement activation by binding the regulatory protein factor H. Proc Natl Acad Sci U S A (2006) 1.96
Dexamethasone induction of hypertension and diabetes is PPAR-alpha dependent in LDL receptor-null mice. Nat Med (2003) 1.94
Lysyl oxidase is required for vascular and diaphragmatic development in mice. J Biol Chem (2002) 1.90
Plasma complement components and activation fragments: associations with age-related macular degeneration genotypes and phenotypes. Invest Ophthalmol Vis Sci (2009) 1.86
Functional rescue of elastin insufficiency in mice by the human elastin gene: implications for mouse models of human disease. Circ Res (2007) 1.83
Implications of the initial mutations in membrane cofactor protein (MCP; CD46) leading to atypical hemolytic uremic syndrome. Mol Immunol (2006) 1.82
CD25 deficiency causes an immune dysregulation, polyendocrinopathy, enteropathy, X-linked-like syndrome, and defective IL-10 expression from CD4 lymphocytes. J Allergy Clin Immunol (2006) 1.81
Rare variants in CFI, C3 and C9 are associated with high risk of advanced age-related macular degeneration. Nat Genet (2013) 1.77
Antagonism of the complement component C4 by flavivirus nonstructural protein NS1. J Exp Med (2010) 1.76
C3-dependent mechanism of microglial priming relevant to multiple sclerosis. Proc Natl Acad Sci U S A (2012) 1.73
Cardiac disease in patients with mucopolysaccharidosis: presentation, diagnosis and management. J Inherit Metab Dis (2011) 1.70
The Pro-regions of lysyl oxidase and lysyl oxidase-like 1 are required for deposition onto elastic fibers. J Biol Chem (2005) 1.69
Vascular respiratory uncoupling increases blood pressure and atherosclerosis. Nature (2005) 1.68
Complement regulator CD46 temporally regulates cytokine production by conventional and unconventional T cells. Nat Immunol (2010) 1.67
Deficiency of tenascin-X causes abnormalities in dermal elastic fiber morphology. J Invest Dermatol (2004) 1.58
Hepatic IL-17 responses in human and murine primary biliary cirrhosis. J Autoimmun (2008) 1.57
Properdin: emerging roles of a pattern-recognition molecule. Annu Rev Immunol (2010) 1.55
Vascular extracellular matrix and aortic development. Curr Top Dev Biol (2004) 1.53
Membrane cofactor protein mutations in atypical hemolytic uremic syndrome (aHUS), fatal Stx-HUS, C3 glomerulonephritis, and the HELLP syndrome. Blood (2007) 1.53
Mutations in complement regulatory proteins predispose to preeclampsia: a genetic analysis of the PROMISSE cohort. PLoS Med (2011) 1.52
DAP12 couples c-Fms activation to the osteoclast cytoskeleton by recruitment of Syk. Mol Cell (2008) 1.52
Reduced vessel elasticity alters cardiovascular structure and function in newborn mice. Circ Res (2009) 1.51
Role of membrane cofactor protein (CD46) in regulation of C4b and C3b deposited on cells. J Immunol (2002) 1.51
Elastic fiber formation: a dynamic view of extracellular matrix assembly using timer reporters. J Cell Physiol (2006) 1.47
Tropoelastin interacts with cell-surface glycosaminoglycans via its COOH-terminal domain. J Biol Chem (2005) 1.46
Upregulation of elastase proteins results in aortic dilatation in mucopolysaccharidosis I mice. Mol Genet Metab (2008) 1.45
Binding of flavivirus nonstructural protein NS1 to C4b binding protein modulates complement activation. J Immunol (2011) 1.45
The application of the iliac artery in the ex vivo reconstruction of renal arteries in renal transplantation. Transplantation (2010) 1.43
Characterization of mutations in complement factor I (CFI) associated with hemolytic uremic syndrome. Mol Immunol (2007) 1.42
The effects of a newly developed miniaturized mechanical chest compressor on outcomes of cardiopulmonary resuscitation in a porcine model*. Crit Care Med (2012) 1.42
Correction of clinical manifestations of canine mucopolysaccharidosis I with neonatal retroviral vector gene therapy. Mol Ther (2007) 1.42
A site on laminin alpha 5, AQARSAASKVKVSMKF, induces inflammatory cell production of matrix metalloproteinase-9 and chemotaxis. J Immunol (2003) 1.38
The elastin-binding protein of Staphylococcus aureus (EbpS) is expressed at the cell surface as an integral membrane protein and not as a cell wall-associated protein. J Biol Chem (2001) 1.37
CD46 is a cellular receptor for all species B adenoviruses except types 3 and 7. J Virol (2005) 1.37
Altered vascular remodeling in fibulin-5-deficient mice reveals a role of fibulin-5 in smooth muscle cell proliferation and migration. Proc Natl Acad Sci U S A (2005) 1.37
Increased fibulin-5 and elastin in S100A4/Mts1 mice with pulmonary hypertension. Circ Res (2005) 1.37
New roles for the major human 3'-5' exonuclease TREX1 in human disease. Cell Cycle (2008) 1.35
CD46: expanding beyond complement regulation. Trends Immunol (2004) 1.32
Fibulin-5 mutations: mechanisms of impaired elastic fiber formation in recessive cutis laxa. Hum Mol Genet (2006) 1.31
The stumbling block in lung repair of emphysema: elastic fiber assembly. Proc Am Thorac Soc (2006) 1.28
A prevalent C3 mutation in aHUS patients causes a direct C3 convertase gain of function. Blood (2012) 1.28
Domains in tropoelastin that mediate elastin deposition in vitro and in vivo. J Biol Chem (2003) 1.27
Elastin haploinsufficiency induces alternative aging processes in the aorta. Rejuvenation Res (2008) 1.26
Deficiency in microfibril-associated glycoprotein-1 leads to complex phenotypes in multiple organ systems. J Biol Chem (2008) 1.26
Upregulation of elastase activity in aorta in mucopolysaccharidosis I and VII dogs may be due to increased cytokine expression. Mol Genet Metab (2009) 1.25
Structure of the C3b binding site of CR1 (CD35), the immune adherence receptor. Cell (2002) 1.25
IL-32: a host proinflammatory factor against influenza viral replication is upregulated by aberrant epigenetic modifications during influenza A virus infection. J Immunol (2010) 1.25
Decay-accelerating factor (DAF), complement receptor 1 (CR1), and factor H dissociate the complement AP C3 convertase (C3bBb) via sites on the type A domain of Bb. J Biol Chem (2001) 1.22
Improvements in mucopolysaccharidosis I mice after adult retroviral vector-mediated gene therapy with immunomodulation. Mol Ther (2007) 1.21
Elastin in large artery stiffness and hypertension. J Cardiovasc Transl Res (2012) 1.21
Structure and regulatory profile of the monkeypox inhibitor of complement: comparison to homologs in vaccinia and variola and evidence for dimer formation. J Immunol (2006) 1.21
Hemophilia gene therapy: a Holy Grail found. Mol Ther (2011) 1.20
New insights into the pathogenesis of autosomal-dominant cutis laxa with report of five ELN mutations. Hum Mutat (2011) 1.20
Neutrophil serine proteinases inactivate surfactant protein D by cleaving within a conserved subregion of the carbohydrate recognition domain. J Biol Chem (2004) 1.18
Discrete contributions of elastic fiber components to arterial development and mechanical compliance. Arterioscler Thromb Vasc Biol (2009) 1.18
Early versus late treatment of spinal cord compression with long-term intrathecal enzyme replacement therapy in canine mucopolysaccharidosis type I. Mol Genet Metab (2010) 1.17
Decreased aortic diameter and compliance precedes blood pressure increases in postnatal development of elastin-insufficient mice. Am J Physiol Heart Circ Physiol (2011) 1.16
The role of the immune response in age-related macular degeneration. Int J Inflam (2013) 1.16
Fibulin-2 is dispensable for mouse development and elastic fiber formation. Mol Cell Biol (2007) 1.16
Interaction of glycoprotein H of human herpesvirus 6 with the cellular receptor CD46. J Biol Chem (2003) 1.16
Emerging roles and new functions of CD46. Springer Semin Immunopathol (2005) 1.16
Elastin protein levels are a vital modifier affecting normal lung development and susceptibility to emphysema. Am J Physiol Lung Cell Mol Physiol (2006) 1.15
Radiographic evaluation of bones and joints in mucopolysaccharidosis I and VII dogs after neonatal gene therapy. Mol Genet Metab (2008) 1.15