X-Linked Alport syndrome with Normal Distribution of Collagen IV α Chains in Epidermal Basement Membrane

1997 ◽  
pp. 134-139 ◽  
Author(s):  
I. Naito ◽  
S. Nomura ◽  
S. Inoue ◽  
M. Kagawa ◽  
T. Matsubara ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Normal Distribution ◽  
Alport Syndrome ◽  
Collagen Iv ◽  
Epidermal Basement Membrane

scholarly journals The Epidermal Basement Membrane Is a Composite of Separate Laminin- or Collagen IV-containing Networks Connected by Aggregated Perlecan, but Not by Nidogens

2012 ◽  
Vol 287 (22) ◽  
pp. 18700-18709 ◽  
Author(s):  
Daniel Timo Behrens ◽  
Daniela Villone ◽  
Manuel Koch ◽  
Georg Brunner ◽  
Lydia Sorokin ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Collagen Iv ◽  
Epidermal Basement Membrane

scholarly journals Epidermal basement membrane α5(IV) expression in females with Alport syndrome and severity of renal disease

2003 ◽  
Vol 64 (5) ◽  
pp. 1787-1791 ◽  
Author(s):  
Laura Massella ◽  
Andrea Onetti Muda ◽  
Tullio Faraggiana ◽  
Cristiano Bette ◽  
Alessandra Renieri ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Renal Disease ◽  
Alport Syndrome ◽  
Epidermal Basement Membrane

scholarly journals Comparison of alpha5(IV) collagen chain expression in skin with disease severity in women with X-linked Alport syndrome.

1998 ◽  
Vol 9 (8) ◽  
pp. 1433-1440
Author(s):  
K Nakanishi ◽  
K Iijima ◽  
N Kuroda ◽  
Y Inoue ◽  
Y Sado ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Renal Disease ◽  
Disease Severity ◽  
End Stage Renal Disease ◽  
Alport Syndrome ◽  
Expression Ratio ◽  
Female Patients ◽  
Stage Renal Disease ◽  
End Stage ◽  
Epidermal Basement Membrane

X-Linked Alport syndrome is caused by mutations in the type IV collagen alpha5 chain gene. Male patients usually develop end-stage renal disease, whereas female patients have more variable phenotypes from asymptomatic hematuria to end-stage renal disease. The variable phenotypes in female patients may be attributable to different X-chromosome inactivation patterns. Therefore, the correlation between disease severity and the degree of alpha5 chain expression in the epidermal basement membrane of female patients with X-linked Alport syndrome was examined. To estimate the disease severity in X-linked Alport syndrome, the ratios of protein to creatinine in single voided urine samples were used. Expression of the alpha5 chain in the epidermal basement membrane was examined by an indirect immunofluorescence method using an anti-alpha5 chain monoclonal antibody. A total of 25 female patients with X-linked Alport syndrome from 17 families was examined. Multiple regression analysis using disease severity as the response variable, and age, family history of nephritis, female and male family history of end-stage renal disease, serum creatinine concentration, and alpha5(IV) expression ratio in the epidermal basement membrane as explanatory variables showed that only alpha5(IV) expression ratio was a significant factor, and that it showed a highly significant negative association with disease severity (adjusted r2=0.71, P=0.0001). These findings suggest that variable alpha5 chain expression, possibly caused by different X-inactivation patterns, is responsible for the variable disease severity in female patients with X-linked Alport syndrome, and that immunohistochemical examination of alpha5 chain expression in the epidermal basement membrane may be a simple and useful method for predicting patient outcome.

scholarly journals Molecular background of hereditary nephropathies in spaniel dogs

2019 ◽  
Vol 75 (12) ◽  
pp. 6330-2019
Author(s):  
MAŁGORZATA WASIELEWSKA ◽  
IWONA SZATKOWSKA ◽  
EWA CZERNIAWSKA–PIĄTKOWSKA ◽  
DANIEL ZABORSKI
Keyword(s):  
Basement Membrane ◽  
Molecular Mechanism ◽  
21St Century ◽  
Glomerular Basement Membrane ◽  
Alport Syndrome ◽  
Collagen Iv ◽  
Gene Coding ◽  
Main Component ◽  
Hereditary Nephropathies

The molecular background of hereditary nephropathies in English Cocker Spaniels and Springer Spaniels remained unclear until the beginning of the 21st century. It was only the discovery of an association between these diseases and Alport syndrome in humans that made it possible to identify the genes potentially responsible for nephropathies in dogs. Eventually, two mutations were identified in the COL4A4 gene coding for the alpha chains of collagen IV, the main component of the glomerular basement membrane (GBM). This review presents the molecular mechanism resulting from the aforementioned mutations, the signs of disease, functions of the GBM, and breeding aspects.

Cardiac myocytes cultured on a basement membrane extract of the ehs tumor

1986 ◽  
Vol 44 ◽  
pp. 270-271
Author(s):  
L. Terracio ◽  
A. Dewey ◽  
K. Rubin ◽  
T.K. Borg
Keyword(s):  
Extracellular Matrix ◽  
Basement Membrane ◽  
Cardiac Myocytes ◽  
Normal Tissue ◽  
Cell Spreading ◽  
Collagen Iv ◽  
Basement Membrane Extract ◽  
Membrane Extract ◽  
Growth Development ◽  
Multicellular Organisms

The recognition and interaction of cells with the extracellular matrix (ECM) effects the normal physiology as well as the pathology of all multicellular organisms. These interactions have been shown to influence the growth, development, and maintenance of normal tissue function. In previous studies, we have shown that neonatal cardiac myocytes specifically interacts with a variety of ECM components including fibronectin, laminin, and collagens I, III and IV. Culturing neonatal myocytes on laminin and collagen IV induces an increased rate of both cell spreading and sarcomerogenesis.

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