Type IV collagen is the major structural component of glomerular basement membranes (GBM), forming a 'chicken-wire' meshwork together with laminins, proteoglycans and entactin/nidogen. Deletions covering the N-terminal regions of COL4A5 and COL4A6, which are localized in a head-to-head manner, are found in the chromosome Xq22.3 centromeric deletion syndrome. This results in a phenotype with features of diffuse leiomyomatosis and Alport syndrome (DL-ATS).
Specifications
Catalog No.
BX50229
Clone No.
BP6206
Application
IHC-P
Subcellular location
basement membrane
Control
Kidney
Recommended method
HIER
Volume
100μl/vial, 1ml/vial
Dilution
1:100-1:200
Immunogen
Recombinant fragment with COL4A5. This information is proprietary to Biolynx and/or its suppliers.
Reference
1. Tsugio Seki et al. Histochem Cell Biol (1998) 110:359-366
2. Yoshioka K et al. Am. J. Pathol (1994) . 144: 986-996.