The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 0.1 - 0.3 µg/ml. Detects a band of approximately 200 kDa (predicted molecular weight: 216 kDa).
Potential cell surface endocytic receptor, which binds and internalizes extracellular ligands for degradation by lysosomes. Involved in the negative regulation of the canonical Wnt signaling pathway, being able to antagonize the LRP6-mediated activation of this pathway.
Expressed in several regions of the brain.
Involvement in disease
Defects in LRP4 are the cause of Cenani-Lenz syndactyly syndrome (CLSS) [MIM:212780]. It is a congenital malformation syndrome defined as complete and complex syndactyly of the hands combined with malformations of the forearm bones and similar manifestations in the lower limbs.
Belongs to the LDLR family. Contains 3 EGF-like domains. Contains 8 LDL-receptor class A domains. Contains 20 LDL-receptor class B repeats.
Selcen D et al. Impaired Synaptic Development, Maintenance, and Neuromuscular Transmission in LRP4-Related Myasthenia. JAMA Neurol72:889-96 (2015).
Read more (PubMed: 26052878) »
Ohkawara B et al. LRP4 third ß-propeller domain mutations cause novel congenital myasthenia by compromising agrin-mediated MuSK signaling in a position-specific manner. Hum Mol GenetN/A:N/A (2013).
Read more (PubMed: 24234652) »