| scholarly article | Q13442814 |
| P2093 | author name string | Thomas N Wight | |
| Aleksander Hinek | |||
| Kathy R Braun | |||
| Kela Liu | |||
| Yanting Wang | |||
| P2860 | cites work | Elastin is an essential determinant of arterial morphogenesis | Q24321276 |
| Impaired elastic-fiber assembly by fibroblasts from patients with either Morquio B disease or infantile GM1-gangliosidosis is linked to deficiency in the 67-kD spliced variant of beta-galactosidase. | Q24538703 | ||
| Versican/PG-M isoforms in vascular smooth muscle cells | Q28139073 | ||
| The proteoglycans aggrecan and Versican form networks with fibulin-2 through their lectin domain binding | Q28141627 | ||
| Expression of PG-M(V3), an alternatively spliced form of PG-M without a chondroitin sulfate attachment in region in mouse and human tissues | Q28235589 | ||
| The 67-kDa enzymatically inactive alternatively spliced variant of beta-galactosidase is identical to the elastin/laminin-binding protein | Q28264251 | ||
| Biological roles of the non-integrin elastin/laminin receptor | Q28296966 | ||
| A novel glycosaminoglycan attachment domain identified in two alternative splice variants of human versican | Q28304691 | ||
| Nature and the multiple functions of the 67-kD elastin-/laminin binding protein | Q28305714 | ||
| A new syndrome: mental subnormality and nasal papillomata | Q28306372 | ||
| Decreased elastin deposition and high proliferation of fibroblasts from Costello syndrome are related to functional deficiency in the 67-kD elastin-binding protein | Q28344006 | ||
| Identification of mutations in the alpha-L-iduronidase gene (IDUA) that cause Hurler and Scheie syndromes | Q28609285 | ||
| Improved retroviral vectors for gene transfer and expression | Q29614384 | ||
| A case of Costello syndrome and glycogen storage disease type III. | Q30495338 | ||
| Isolation of soluble elastin-lathyrism | Q70353150 | ||
| Enhancing the efficiency of DNA-mediated gene transfer in mammalian cells | Q70958753 | ||
| Functional interplay between interleukin-1 receptor and elastin binding protein regulates fibronectin production in coronary artery smooth muscle cells | Q71090172 | ||
| Coronary artery disease in the Hurler syndrome. Qualitative and quantitative analysis of the extent of coronary narrowing at necropsy in six children | Q71536189 | ||
| Growth stimulation of human skin fibroblasts by elastin-derived peptides | Q71567081 | ||
| Cell cycle analysis of a cell proliferation-associated human nuclear antigen defined by the monoclonal antibody Ki-67 | Q72743242 | ||
| Cardiovascular changes in children with mucopolysaccharide storage diseases and related disorders--clinical and echocardiographic findings in 64 patients | Q74827766 | ||
| Versican interacts with fibrillin-1 and links extracellular microfibrils to other connective tissue networks | Q77300620 | ||
| Screening for cancer in children with Costello syndrome | Q77667535 | ||
| Retrovirally mediated overexpression of versican v3 by arterial smooth muscle cells induces tropoelastin synthesis and elastic fiber formation in vitro and in neointima after vascular injury | Q77743543 | ||
| Glycosaminoglycan accumulation and excretion in the mucopolysaccharidoses: characterization and basis of a diagnostic test for MPS | Q77795825 | ||
| An infant with Costello syndrome complicated with fatal hypertrophic obstructive cardiomyopathy | Q77808161 | ||
| Elastin: molecular description and function | Q33601688 | ||
| Impaired elastogenesis in Hurler disease: dermatan sulfate accumulation linked to deficiency in elastin-binding protein and elastic fiber assembly | Q33892206 | ||
| Costello syndrome: a cancer predisposing syndrome? | Q34068412 | ||
| Five additional Costello syndrome patients with rhabdomyosarcoma: proposal for a tumor screening protocol | Q34115295 | ||
| Elastic fibres | Q34135146 | ||
| Impaired elastin fiber assembly related to reduced 67-kD elastin-binding protein in fetal lamb ductus arteriosus and in cultured aortic smooth muscle cells treated with chondroitin sulfate | Q34216561 | ||
| Further delineation of cardiac abnormalities in Costello syndrome | Q34811131 | ||
| Versican: a versatile extracellular matrix proteoglycan in cell biology | Q34858300 | ||
| Costello syndrome: orthopaedic manifestations and functional health | Q35033683 | ||
| Costello syndrome: an overview | Q35057638 | ||
| Connection between elastin haploinsufficiency and increased cell proliferation in patients with supravalvular aortic stenosis and Williams-Beuren syndrome | Q37362266 | ||
| The elastin receptor: a galactoside-binding protein | Q38347627 | ||
| The 67-kD elastin/laminin-binding protein is related to an enzymatically inactive, alternatively spliced form of beta-galactosidase | Q40303835 | ||
| A critical role for elastin signaling in vascular morphogenesis and disease | Q40685668 | ||
| Cell-mediated transfer of proteoglycan genes | Q40792980 | ||
| Genotype-phenotype correlations in mucopolysaccharidosis type I using enzyme kinetics, immunoquantification and in vitro turnover studies. | Q41006881 | ||
| Molecular genetics of mucopolysaccharidosis type I: diagnostic, clinical, and biological implications. | Q41015480 | ||
| Life-threatening cardiac involvement throughout life in a case of Costello syndrome | Q41321455 | ||
| Cardiac disease in Costello syndrome | Q41729292 | ||
| Recycling of the 67-kDa elastin binding protein in arterial myocytes is imperative for secretion of tropoelastin | Q41932422 | ||
| Cutis laxa: a feature of Costello syndrome | Q41974596 | ||
| Cell cycle-dependent regulation of elastin gene in cultured chick vascular smooth-muscle cells | Q42160528 | ||
| 67-kD elastin-binding protein is a protective "companion" of extracellular insoluble elastin and intracellular tropoelastin | Q42953033 | ||
| Studies on the pathogenesis of Costello syndrome | Q43074208 | ||
| Hypertrophic cardiomyopathy in mucopolysaccharidoses: regression after bone marrow transplantation | Q43409073 | ||
| Overexpression of the V3 variant of versican alters arterial smooth muscle cell adhesion, migration, and proliferation in vitro. | Q43864458 | ||
| Signaling pathways transduced through the elastin receptor facilitate proliferation of arterial smooth muscle cells | Q44147048 | ||
| Polyclonal antibodies to tropoelastin and the specific detection and measurement of tropoelastin in vitro. | Q44273851 | ||
| Structural changes and facilitated association of tropoelastin | Q44306047 | ||
| The heart in the Hurler syndrome: gross, histologic and ultrastructural observations in five necropsy cases | Q44589814 | ||
| Costello syndrome: phenotype, natural history, differential diagnosis, and possible cause. | Q45946323 | ||
| Multiple forms of mouse PG-M, a large chondroitin sulfate proteoglycan generated by alternative splicing | Q48075592 | ||
| Fibulin-1 is a ligand for the C-type lectin domains of aggrecan and versican | Q48167246 | ||
| Tropoelastin and elastin degradation products promote proliferation of human astrocytoma cell lines | Q48465175 | ||
| Diagnostic test for mucopolysaccharidosis. I. Direct method for quantifying excessive urinary glycosaminoglycan excretion. | Q50889906 | ||
| Further delineation of the behavioral and neurologic features in Costello syndrome. | Q51950345 | ||
| Costello syndrome in two siblings and minor manifestations in their mother. Further evidence for autosomal dominant inheritance? | Q51952405 | ||
| Elastic fiber degeneration in Costello syndrome. | Q52009394 | ||
| Pathology of the elastic tissue of the skin in Costello syndrome. An image analysis study using mathematical morphology. | Q52364979 | ||
| Domain 26 of tropoelastin plays a dominant role in association by coacervation. | Q52539692 | ||
| Resting Tl-201 scintigraphy in the evaluation of coronary artery disease in children with Hurler syndrome. | Q52880456 | ||
| Hurler syndrome: past, present, and future. | Q53572902 | ||
| Exclusion of the elastin gene in the pathogenesis of Costello syndrome | Q58923994 | ||
| Vascular smooth muscle cell detachment from elastin and migration through elastic laminae is promoted by chondroitin sulfate-induced "shedding" of the 67-kDa cell surface elastin binding protein | Q67507691 | ||
| Hurler's syndrome, an -L-iduronidase deficiency | Q68491649 | ||
| Hurler syndrome with cardiomyopathy in infancy | Q69180093 | ||
| The protein components of the 12-nanometer microfibrils of elastic and nonelastic tissues | Q69490024 | ||
| P433 | issue | 1 | |
| P407 | language of work or name | English | Q1860 |
| P921 | main subject | patient | Q181600 |
| Costello syndrome | Q1136492 | ||
| overexpression | Q61643320 | ||
| P304 | page(s) | 119-131 | |
| P577 | publication date | 2004-01-01 | |
| P1433 | published in | The American Journal of Pathology | Q4744259 |
| P1476 | title | Retrovirally mediated overexpression of versican v3 reverses impaired elastogenesis and heightened proliferation exhibited by fibroblasts from Costello syndrome and Hurler disease patients | |
| P478 | volume | 164 |
| Q36672030 | ADAMTS-1 and ADAMTS-4 levels are elevated in thoracic aortic aneurysms and dissections |
| Q33781787 | Alteration of chondroitin sulfate composition on proteoglycan produced by knock-in mouse embryonic fibroblasts whose versican lacks the A subdomain |
| Q34883690 | Arterial pathology in canine mucopolysaccharidosis-I and response to therapy |
| Q39654702 | C4ST-1/CHST11-controlled chondroitin sulfation interferes with oncogenic HRAS signaling in Costello syndrome |
| Q36678349 | Changes in elastin, elastin binding protein and versican in alveoli in chronic obstructive pulmonary disease |
| Q36713856 | Chondroitin sulfate as a key molecule in the development of atherosclerosis and cancer progression |
| Q36703894 | Cutaneous manifestations of Costello syndrome |
| Q49484206 | Decellularization and Solubilization of Porcine Liver for Use as a Substrate for Porcine Hepatocyte Culture: Method Optimization and Comparison |
| Q46723041 | Expression and localization of versican during postnatal development of rat temporomandibular joint disc |
| Q36065126 | Expression of V3 Versican by Rat Arterial Smooth Muscle Cells Promotes Differentiated and Anti-inflammatory Phenotypes |
| Q34075918 | Expression of versican V3 by arterial smooth muscle cells alters tumor growth factor β (TGFβ)-, epidermal growth factor (EGF)-, and nuclear factor κB (NFκB)-dependent signaling pathways, creating a microenvironment that resists monocyte adhesion |
| Q33617665 | Expression of versican isoform V3 in the absence of ascorbate improves elastogenesis in engineered vascular constructs |
| Q24634333 | Fibulin-4 regulates expression of the tropoelastin gene and consequent elastic-fibre formation by human fibroblasts |
| Q36954963 | G1 Domain of Versican Regulates Hyaluronan Organization and the Phenotype of Cultured Human Dermal Fibroblasts |
| Q36431974 | Human iPS Cell-Derived Neurons Uncover the Impact of Increased Ras Signaling in Costello Syndrome |
| Q36730629 | Inhibition of versican expression by siRNA facilitates tropoelastin synthesis and elastic fiber formation by human SK-LMS-1 leiomyosarcoma smooth muscle cells in vitro and in vivo |
| Q35022323 | Insight into pathologic abnormalities in congenital semilunar valve disease based on advances in understanding normal valve microstructure and extracellular matrix |
| Q35069763 | Neointima formed by arterial smooth muscle cells expressing versican variant V3 is resistant to lipid and macrophage accumulation |
| Q38318365 | Primary and secondary elastin-binding protein defect leads to impaired elastogenesis in fibroblasts from GM1-gangliosidosis patients. |
| Q37295537 | Pro-inflammatory phenotype of COPD fibroblasts not compatible with repair in COPD lung |
| Q50220949 | Proteoglycan expression is influenced by mechanical load in TMJ discs |
| Q39033619 | Provisional matrix: A role for versican and hyaluronan |
| Q35378176 | Retinoblastoma protein modulates the inverse relationship between cellular proliferation and elastogenesis |
| Q37061141 | Retrovirally mediated overexpression of glycosaminoglycan-deficient biglycan in arterial smooth muscle cells induces tropoelastin synthesis and elastic fiber formation in vitro and in neointimae after vascular injury |
| Q34401134 | The Hyperthermia-enhanced Association between Tropoelastin and Its 67-kDa Chaperone Results in Better Deposition of Elastic Fibers |
| Q37627561 | The bladder extracellular matrix. Part I: architecture, development and disease |
| Q38299267 | The intracellular form of human MAGP1 elicits a complex and specific transcriptional response |
| Q81018390 | The mild form of mucopolysaccharidosis type I (Scheie syndrome) is associated with increased ascending aortic stiffness |
| Q37059193 | The mucopolysaccharidoses: a success of molecular medicine. |
| Q41697451 | The role of elastic fibers in pathogenesis of conjunctivochalasis |
| Q89452415 | The synthesis and secretion of versican isoform V3 by mammalian cells: A role for N-linked glycosylation |
| Q36190676 | Tumor predisposition in Costello syndrome |
| Q53521965 | Use of versican variant V3 and versican antisense expression to engineer cultured human skin containing increased content of insoluble elastin. |
| Q45220723 | V3 versican isoform expression alters the phenotype of melanoma cells and their tumorigenic potential. |
| Q33690423 | Versican and the control of inflammation |
| Q27690926 | Versican and the regulation of cell phenotype in disease |
| Q43158580 | Versican/PG-M Assembles Hyaluronan into Extracellular Matrix and Inhibits CD44-mediated Signaling toward Premature Senescence in Embryonic Fibroblasts |
| Q27354400 | Visualizing tropoelastin in a long-term human elastic fibre cell culture model |
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