Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The Lutheran blood group glycoprotein (Lu), also known as basal cell adhesion molecule, is an Ig superfamily transmembrane receptor for laminin alpha5. Lu is expressed on the surface of a subset of muscle and epithelial cells in diverse tissues and is thought to be involved in both normal and disease processes, including sickle cell disease and cancer. Here we investigated the binding of Lu to laminin alpha5 in vivo and in vitro. We prepared a soluble recombinant Lu (sol-Lu) composed of the Lu extracellular domain and a His(6) tag. Sol-Lu bound specifically to laminin-10/11 (alpha5beta1/beta2gamma1) in enzyme-linked immunosorbent assays and bound to bona fide basement membranes containing laminin alpha5 in tissue sections. Sol-Lu did not bind to tissue sections of laminin alpha5 knockout embryos, despite the fact that the four other alpha chains were present. To identify the Lu-binding site on laminin alpha5, we prepared modified alpha5 cDNAs encoding chimeric laminins containing all or part of the laminin alpha1 G domain in place of the analogous alpha5 regions. These constructs were used to generate transgenic mice. Proteins derived from transgenes were detected in basement membranes and were assayed for their ability to bind Lu by examining the localization of endogenous Lu and the binding of sol-Lu applied to tissue sections. Our results demonstrate that the alpha5 LG3 module is essential for Lu binding to laminin alpha5.
 ...
PMID:Identification of the binding site for the Lutheran blood group glycoprotein on laminin alpha 5 through expression of chimeric laminin chains in vivo. 1224 66

The Lutheran blood group glycoprotein (Lu), also known as basal cell adhesion molecule (B-CAM), is a transmembrane receptor with five immunoglobulin-like domains in its extracellular region; it is therefore classified as a member of the immunoglobulin (Ig) gene family. Lu/B-CAM is observed not only on red blood cells, but also on a subset of muscle and epithelial cells in various tissues. Recently, several groups have reported that Lu/B-CAM is a novel receptor for laminin a5. The laminin a5 chain is a component of the laminin-511 (alpha 5 beta 1 gamma 1), -521 (alpha 5 beta 2 gamma 1), and -523 (alpha 5 beta 2 gamma 3) heterotrimers and is expressed throughout the mammalian body. We also have shown that Lu/B-CAM is co-localized with laminin alpha 5 in various tissues. Although the biological role of Lu/B-CAM remains unclear, the specific binding of Lu/B-CAM to laminin alpha 5 suggests that it plays an important role in developmental and physiological processes. It also is necessary to investigate further the interaction between Lu/B-CAM and laminin a5 in pathological processes, including sickle cell disease and cancer.
 ...
PMID:Review: Lutheran/B-CAM: a laminin receptor on red blood cells and in various tissues. 1654 22