The Lutheran glycoprotein: a multifunctional adhesion receptor.
The Lutheran blood group system, which comprises one of the largest families of human red blood cell (RBC) antigens, resides on two immunoglobulin superfamily (IgSF) proteins: Lutheran and basal cell adhesion molecule (B-CAM). These two glycoproteins arise via alternative splicing of mRNA from a single gene and differ in structure only in the lengths of their cytoplasmic tails. Both are expressed on RBCs as well as a variety of other cell types, and they are overexpressed on sickle RBCs (SS RBC). B-CAM/Lu is the critical receptor for SS RBC adhesion to the extracellular matrix protein laminin, an interaction thought to contribute to the pathogenesis of sickle cell-related vasoocclusive events. Recent work has also shown that B-CAM/Lu on RBCs can undergo activation as a result of adrenergic signaling pathways. The high affinity of B-CAM/Lu for laminin is also thought to contribute to various developmental processes, including organogenesis, vascular development, erythropoiesis, and smooth muscle development and organization. Interestingly, the B-CAM spliceoform seems to be overexpressed by a variety of different malignant tumors and may be involved, along with other adhesion receptor proteins, in malignant transformation and tumor metastasis. Studies of B-CAM/Lu have thus expanded from defining antigen-specific polymorphisms to investigations of processes involved in sickle cell disease, human development, and cancer biology.
Eyler
Christine E
CE
Duke Comprehensive Sickle Cell Center and Division of Hematology, Department of Medicine, Duke University, Durham, NC 27710, USA.
Telen
Marilyn J
MJ
D000595:Amino Acid Sequence; D015703:Antigens, CD / Q000235:genetics / Q000502:physiology; D000945:Antigens, Differentiation, T-Lymphocyte / Q000235:genetics / Q000502:physiology; D001483:Base Sequence; D002448:Cell Adhesion / Q000502:physiology*; D004912:Erythrocytes / Q000166:cytology / Q000502:physiology; D005786:Gene Expression Regulation; D005787:Gene Frequency; D006801:Humans; D037181:Lectins, C-Type; D008188:Lutheran Blood-Group System / Q000235:genetics / Q000502:physiology*; D008562:Membrane Glycoproteins; D008565:Membrane Proteins / Q000502:physiology*; D008969:Molecular Sequence Data; D019038:Platelet Glycoprotein GPIb-IX Complex; D000099192:CD69 Antigens
D015703:Antigens, CD; D000945:Antigens, Differentiation, T-Lymphocyte; D037181:Lectins, C-Type; D008188:Lutheran Blood-Group System; D008562:Membrane Glycoproteins; D008565:Membrane Proteins; D019038:Platelet Glycoprotein GPIb-IX Complex; D000099192:CD69 Antigens; C094990:adhesion receptor