Alport syndrome: its effects on the glomerular filtration barrier and implications for future treatment (Q38238367)

31 August 2017

title

Alport syndrome: its effects on the glomerular filtration barrier and implications for future treatment (English)

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31 August 2017

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Judy Savige

1

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language of work or name

English

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publication date

8 August 2014

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31 August 2017

published in

Journal of Physiology

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31 August 2017

volume

592

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31 August 2017

issue

18

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31 August 2017

page(s)

4013-4023

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Cellular origins of type IV collagen networks in developing glomeruli.

31 August 2017

cites work

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Preemptive ramipril therapy delays renal failure and reduces renal fibrosis in COL4A3-knockout mice with Alport syndrome

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Early angiotensin-converting enzyme inhibition in Alport syndrome delays renal failure and improves life expectancy

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Revisiting the glomerular charge barrier in the molecular era.

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Electrical Forces Determine Glomerular Permeability

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Correlation of histopathological features and renal impairment in autosomal dominant Alport syndrome in Bull terriers

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Isoform switching of type IV collagen is developmentally arrested in X-linked Alport syndrome leading to increased susceptibility of renal basement membranes to endoproteolysis

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Identification of variant Alport phenotypes using an Alport-specific antibody probe

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Irradiation prolongs survival of Alport mice

21 January 2018

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Positionally cloned gene for a novel glomerular protein--nephrin--is mutated in congenital nephrotic syndrome

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Alport syndrome--insights from basic and clinical research

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Stem cell therapies benefit Alport syndrome

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The COOH terminus of megalin regulates gene expression in opossum kidney proximal tubule cells

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Biomechanical strain causes maladaptive gene regulation, contributing to Alport glomerular disease

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Laminin functions in tissue morphogenesis.

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Delayed chemokine receptor 1 blockade prolongs survival in collagen 4A3-deficient mice with Alport disease.

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An update on the pathomechanisms and future therapies of Alport syndrome

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Mapping structural landmarks, ligand binding sites, and missense mutations to the collagen IV heterotrimers predicts major functional domains, novel interactions, and variation in phenotypes in inherited diseases affecting basement membranes.

21 January 2018

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A novel surgical organ perfusion method for effective ex vivo and in vivo gene transfer into renal glomerular cells

21 January 2018

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Evidence for activation of the unfolded protein response in collagen IV nephropathies

21 January 2018

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Bone marrow-derived cells contribute to podocyte regeneration and amelioration of renal disease in a mouse model of Alport syndrome.

21 January 2018

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Role for macrophage metalloelastase in glomerular basement membrane damage associated with alport syndrome

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FcRn: the neonatal Fc receptor comes of age.

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Expert guidelines for the management of Alport syndrome and thin basement membrane nephropathy.

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Thin basement membrane nephropathy

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Role for transforming growth factor-beta1 in alport renal disease progression

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An orally active chemokine receptor CCR2 antagonist prevents glomerulosclerosis and renal failure in type 2 diabetes

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Multiple metabolic hits converge on CD36 as novel mediator of tubular epithelial apoptosis in diabetic nephropathy

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Loss of the BMP antagonist USAG-1 ameliorates disease in a mouse model of the progressive hereditary kidney disease Alport syndrome.

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C3a mediates epithelial-to-mesenchymal transition in proteinuric nephropathy

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A sulfilimine bond identified in collagen IV

21 January 2018

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Discoidin domain receptor 1 is activated independently of beta(1) integrin

21 January 2018

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The discoidin domain receptor tyrosine kinases are activated by collagen

21 January 2018

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https://api.crossref.org/works/10.1113/JPHYSIOL.2014.274449

Nephrin strands contribute to a porous slit diaphragm scaffold as revealed by electron tomography

21 January 2018

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https://api.crossref.org/works/10.1113/JPHYSIOL.2014.274449

Biophysical properties of normal and diseased renal glomeruli

21 January 2018

1 reference

https://api.crossref.org/works/10.1113/JPHYSIOL.2014.274449

α1β1 integrin/Rac1-dependent mesangial invasion of glomerular capillaries in Alport syndrome

21 January 2018

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https://api.crossref.org/works/10.1113/JPHYSIOL.2014.274449

Stage-specific action of matrix metalloproteinases influences progressive hereditary kidney disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1113/JPHYSIOL.2014.274449

Feasibility of repairing glomerular basement membrane defects in Alport syndrome

21 January 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4198011

Glomerular size and charge selectivity in the rat as revealed by FITC-ficoll and albumin

1 October 2018

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4198011

X-linked Alport syndrome: natural history in 195 families and genotype- phenotype correlations in males

1 October 2018

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4198011

Adenovirus-mediated gene transfer into kidney glomeruli using an ex vivo and in vivo kidney perfusion system - first steps towards gene therapy of Alport syndrome.

1 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4198011

The alpha chains of type IV collagen.

1 October 2018

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4198011

Glomerular basement membrane. Identification of a novel disulfide-cross-linked network of alpha3, alpha4, and alpha5 chains of type IV collagen and its implications for the pathogenesis of Alport syndrome

1 October 2018

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12 December 2020

[AN UNUSUAL CONGENITAL AND FAMILIAL CONGENITAL MALFORMATIVE COMBINATION INVOLVING THE EYE AND KIDNEY.]

1 reference

12 December 2020

Nephroprotective effect of the HMG-CoA-reductase inhibitor cerivastatin in a mouse model of progressive renal fibrosis in Alport syndrome

1 reference

12 December 2020

Laminin compensation in collagen alpha3(IV) knockout (Alport) glomeruli contributes to permeability defects

1 reference

12 December 2020

Inhibition of NF-kappaB-dependent Bcl-xL expression by clusterin promotes albumin-induced tubular cell apoptosis

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12 December 2020

Resolved: normal glomeruli filter nephrotic levels of albumin

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12 December 2020

Morphological and functional evidence for an important role of the endothelial cell glycocalyx in the glomerular barrier

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12 December 2020

Fenestrated glomerular capillaries are unique

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12 December 2020

The R229Q mutation in NPHS2 may predispose to proteinuria in thin-basement-membrane nephropathy

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12 December 2020

Red cell traverse through thin glomerular basement membranes

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12 December 2020

The molecular basis of Goodpasture and Alport syndromes: beacons for the discovery of the collagen IV family

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12 December 2020

Bacterial CpG-DNA accelerates Alport glomerulosclerosis by inducing an M1 macrophage phenotype and tumor necrosis factor-α-mediated podocyte loss

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12 December 2020

Tubular toxicity of proteinuria

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12 December 2020

10.1113/JPHYSIOL.2014.274449

Identifiers

DOI

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31 August 2017

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