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).
機能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.
組織特異性Expressed in several regions of the brain.
関連疾患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.
配列類似性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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