货品编号: 博奥森.bs-6435R
品牌: 博奥森
品名: 肾病样蛋白1抗体
规格: 100u
产品描述:
Neph1(Kirrel) belongs to a family of three closely related transmembrane proteins of the Ig superfamily with a structure similar to that of nephrin. Neph1 consists of a signal peptide, five Ig-like C2-type domains with the middle domain overlapping with a PKD-like domain, an RGD sequence, a transmembrane domain and a cytoplasmic tail. Neph1 is abundantly expressed in the kidney, specifically expressed in podocytes of kidney glomeruli, and plays a significant role in the normal development and function of the glomerular permeability. Neph1 interacts with nephrin in vitro and in vivo, and able to stimulate transcriptional activation in a model system, such as the activation the transcription factor AP-1 via the stimulation of a MAPK module. Neph1 is crucial for the integrity of the slit diaphragm, as Neph1 gene knockout mice results in effacement of glomerular podocytes, heavy proteinuria, and early postnatal death. .
Tissue specificity:
Abundantly expressed in kidney. Specifically expressed in podocytes of kidney glomeruli.
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