Alpha-actinin-4-mediated FSGS: an inherited kidney disease caused by an aggregated and rapidly degraded cytoskeletal protein
about
Synaptopodin regulates the actin-bundling activity of alpha-actinin in an isoform-specific mannerFamilial focal segmental glomerulosclerosis (FSGS)-linked α-actinin 4 (ACTN4) protein mutants lose ability to activate transcription by nuclear hormone receptorsα-Actinin 4 potentiates nuclear factor κ-light-chain-enhancer of activated B-cell (NF-κB) activity in podocytes independent of its cytoplasmic actin binding functionACTN1 mutations cause congenital macrothrombocytopeniaTRPC6 in glomerular health and disease: what we know and what we believeThe Impact of Histologic Variants on FSGS OutcomesCrystal Structure of the Actin-Binding Domain of α-Actinin-4 Lys255Glu Mutant Implicated in Focal Segmental GlomerulosclerosisTranslating genetic findings in hereditary nephrotic syndrome: the missing loopsDisruption of Myosin 1e promotes podocyte injuryα-Actinin-4/FSGS1 is required for Arp2/3-dependent actin assembly at the adherens junctionPodocyte-associated talin1 is critical for glomerular filtration barrier maintenance.Discovery of new glomerular disease-relevant genes by translational profiling of podocytes in vivo.Pathophysiology and treatment of focal segmental glomerulosclerosis: the role of animal models.Genetic causes of proteinuria and nephrotic syndrome: impact on podocyte pathobiology.Alpha-actinin-4 and CLP36 protein deficiencies contribute to podocyte defects in multiple human glomerulopathies.A novel missense mutation of Wilms' Tumor 1 causes autosomal dominant FSGS.Pharmacological targeting of actin-dependent dynamin oligomerization ameliorates chronic kidney disease in diverse animal models.α-Actinin-2 deficiency results in sarcomeric defects in zebrafish that cannot be rescued by α-actinin-3 revealing functional differences between sarcomeric isoforms.Disease-associated mutant alpha-actinin-4 reveals a mechanism for regulating its F-actin-binding affinity.Functional Validation of an Alpha-Actinin-4 Mutation as a Potential Cause of an Aggressive Presentation of Adolescent Focal Segmental Glomerulosclerosis: Implications for Genetic Testing.Podocyte biology in human disease.Clinical impact of research on the podocyte slit diaphragm.Pathophysiology of focal segmental glomerulosclerosis.Patients with ACTN4 mutations demonstrate distinctive features of glomerular injury.Chapter 19: Mechanical response of cytoskeletal networks.Podocyte actin in health, disease and treatment.The origin and pathogenic consequences of anti-dsDNA antibodies in systemic lupus erythematosus.Pathogenesis and therapy of focal segmental glomerulosclerosis: an update.Podosomal proteins as causes of human syndromes: a role in craniofacial development?Educational paper: the podocytopathies.Relevance of the ACTN4 gene in African-Americans with non-diabetic end-stage renal disease.The role of alpha-actinin-4 in human kidney disease.α Actinin 4 (ACTN4) Regulates Glucocorticoid Receptor-mediated Transactivation and Transrepression in Podocytes.The podocyte cytoskeleton in health and in disease.Three-layered proteomic characterization of a novel ACTN4 mutation unravels its pathogenic potential in FSGS.Bigenic mouse models of focal segmental glomerulosclerosis involving pairwise interaction of CD2AP, Fyn, and synaptopodin.TRPC6 G757D Loss-of-Function Mutation Associates with FSGS.Phylogenetic analysis of gene structure and alternative splicing in alpha-actinins.Mice with altered alpha-actinin-4 expression have distinct morphologic patterns of glomerular disease.Podocyte Injury Associated with Mutant α-Actinin-4
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P2860
Alpha-actinin-4-mediated FSGS: an inherited kidney disease caused by an aggregated and rapidly degraded cytoskeletal protein
description
2004 nî lūn-bûn
@nan
2004 թուականի Յունիսին հրատարակուած գիտական յօդուած
@hyw
2004 թվականի հունիսին հրատարակված գիտական հոդված
@hy
2004年の論文
@ja
2004年論文
@yue
2004年論文
@zh-hant
2004年論文
@zh-hk
2004年論文
@zh-mo
2004年論文
@zh-tw
2004年论文
@wuu
name
Alpha-actinin-4-mediated FSGS: ...... degraded cytoskeletal protein
@ast
Alpha-actinin-4-mediated FSGS: ...... degraded cytoskeletal protein
@en
Alpha-actinin-4-mediated FSGS: ...... degraded cytoskeletal protein
@nl
type
label
Alpha-actinin-4-mediated FSGS: ...... degraded cytoskeletal protein
@ast
Alpha-actinin-4-mediated FSGS: ...... degraded cytoskeletal protein
@en
Alpha-actinin-4-mediated FSGS: ...... degraded cytoskeletal protein
@nl
prefLabel
Alpha-actinin-4-mediated FSGS: ...... degraded cytoskeletal protein
@ast
Alpha-actinin-4-mediated FSGS: ...... degraded cytoskeletal protein
@en
Alpha-actinin-4-mediated FSGS: ...... degraded cytoskeletal protein
@nl
P2093
P2860
P1433
P1476
Alpha-actinin-4-mediated FSGS: ...... degraded cytoskeletal protein
@en
P2093
Bradley M Denker
Claudine H Kos
Martin R Pollak
Phillip G Allen
P2860
P356
10.1371/JOURNAL.PBIO.0020167
P407
P577
2004-06-15T00:00:00Z