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).
功能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.
组织特异性Expressed in several regions of the brain.
疾病相关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.
序列相似性Belongs to the LDLR family. Contains 3 EGF-like domains. Contains 8 LDL-receptor class A domains. Contains 20 LDL-receptor class B repeats.
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).
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