Formation and progression of sub-retinal pigment epithelium deposits in Efemp1 mutation knock-in mice: a model for the early pathogenic course of macular degeneration.
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Comparison of an expanded ataxia interactome with patient medical records reveals a relationship between macular degeneration and ataxiaEFEMP1 binds the EGF receptor and activates MAPK and Akt pathways in pancreatic carcinoma cellsThe proteomics of drusenAnimal models of age related macular degenerationFibulin-3 is uniquely upregulated in malignant gliomas and promotes tumor cell motility and invasionDisruption of murine Adamtsl4 results in zonular fiber detachment from the lens and in retinal pigment epithelium dedifferentiationThe Project MACULA Retinal Pigment Epithelium Grading System for Histology and Optical Coherence Tomography in Age-Related Macular DegenerationExome Sequencing Identifies a Missense Variant in EFEMP1 Co-Segregating in a Family with Autosomal Dominant Primary Open-Angle Glaucoma.Extreme retinal remodeling triggered by light damage: implications for age related macular degeneration.Early AMD-like defects in the RPE and retinal degeneration in aged mice with RPE-specific deletion of Atg5 or Atg7Fibulin 2, a tyrosine O-sulfated protein, is up-regulated following retinal detachment.Cholesterol in the retina: the best is yet to come.Retinal ultrastructure of murine models of dry age-related macular degeneration (AMD).Mutant Fibulin-3 Causes Proteoglycan Accumulation and Impaired Diffusion Across Bruch's Membrane.A novel haplotype with the R345W mutation in the EFEMP1 gene associated with autosomal dominant drusen in a Japanese family.Focus on molecules: fibulin-3 (EFEMP1).Changes in fibroblast mechanostat set point and mechanosensitivity: an adaptive response to mechanical stress in floppy eyelid syndromeA high-throughput cell-based Gaussia luciferase reporter assay for identifying modulators of fibulin-3 secretionInteraction of complement factor h and fibulin3 in age-related macular degenerationA CTRP5 gene S163R mutation knock-in mouse model for late-onset retinal degenerationGenetic ablation of N-linked glycosylation reveals two key folding pathways for R345W fibulin-3, a secreted protein associated with retinal degeneration.A comparison of some organizational characteristics of the mouse central retina and the human maculaCompromised mutant EFEMP1 secretion associated with macular dystrophy remedied by proteostasis network alteration.A chimeric Cfh transgene leads to increased retinal oxidative stress, inflammation, and accumulation of activated subretinal microglia in miceNatural history of age-related retinal lesions that precede AMD in mice fed high or low glycemic index diets.Differential tolerance of 'pseudo-pathogenic' tryptophan residues in calcium-binding EGF domains of short fibulin proteins.A local complement response by RPE causes early-stage macular degeneration.Deletion of myosin VI causes slow retinal optic neuropathy and age-related macular degeneration (AMD)-relevant retinal phenotype.Absence of DJ-1 causes age-related retinal abnormalities in association with increased oxidative stress.Deletion of Efemp1 Is Protective Against the Development of Sub-RPE Deposits in Mouse Eyes.Unraveling a multifactorial late-onset disease: from genetic susceptibility to disease mechanisms for age-related macular degeneration.Translational attenuation differentially alters the fate of disease-associated fibulin proteinsCopy-number disorders are a common cause of congenital kidney malformationsTargeting of exon VI-skipping human RGR-opsin to the plasma membrane of pigment epithelium and co-localization with terminal complement complex C5b-9Retinal microstructure in patients with EFEMP1 retinal dystrophy evaluated by Fourier domain OCT.Mouse genetics and proteomic analyses demonstrate a critical role for complement in a model of DHRD/ML, an inherited macular degeneration.Subretinal drusenoid deposits in non-neovascular age-related macular degeneration: morphology, prevalence, topography, and biogenesis model.Protein misfolding and retinal degenerationRisk factors and biomarkers of age-related macular degeneration.Mapping wild-type and R345W fibulin-3 intracellular interactomes.
P2860
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P2860
Formation and progression of sub-retinal pigment epithelium deposits in Efemp1 mutation knock-in mice: a model for the early pathogenic course of macular degeneration.
description
2007 nî lūn-bûn
@nan
2007年の論文
@ja
2007年学术文章
@wuu
2007年学术文章
@zh
2007年学术文章
@zh-cn
2007年学术文章
@zh-hans
2007年学术文章
@zh-my
2007年学术文章
@zh-sg
2007年學術文章
@yue
2007年學術文章
@zh-hant
name
Formation and progression of s ...... ourse of macular degeneration.
@en
Formation and progression of s ...... ourse of macular degeneration.
@nl
type
label
Formation and progression of s ...... ourse of macular degeneration.
@en
Formation and progression of s ...... ourse of macular degeneration.
@nl
prefLabel
Formation and progression of s ...... ourse of macular degeneration.
@en
Formation and progression of s ...... ourse of macular degeneration.
@nl
P2093
P356
P1476
Formation and progression of s ...... ourse of macular degeneration.
@en
P2093
Alan D Marmorstein
Lihua Y Marmorstein
Neal S Peachey
Precious J McLaughlin
Takako Sasaki
P304
P356
10.1093/HMG/DDM199
P577
2007-07-30T00:00:00Z