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- bs-41299P
- 2025年10月16日
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- 规格：
100ug/500ug/20ug
| 规格： | 100ug | 产品价格： | ￥1880.0 |
|---|---|---|---|
| 规格： | 500ug | 产品价格： | ￥5680.0 |
| 规格： | 20ug | 产品价格： | ￥580.0 |
| 产品编号 | bs-41299P |
| 英文名称 | Recombinant human Nephrin, His |
| 中文名称 | 重组人肾小球细胞粘附分子受体蛋白 |
| 英文别名 | CNF; NPHN; Nephrosis 1 congenital Finnish type; NPHS 1; NPHS1; Renal glomerulus specific cell adhesion receptor; Renal glomerulus-specific cell adhesion receptor; NPHN_HUMAN ; Recombinant human Nephrin, His |
| 性状 | Lyophilized or Liqui |
| 纯化方法 | AC |
| 理论分子量 | 30.5 |
| 浓度 | ＞0.5 mg/ml |
| 研究领域 | Signal Transduction > Cytoskeleton / ECM > Cell Adhesion > Cell Adhesion Molecules > Endothelial |
| 亚基 | Interacts with CD2AP (via C-terminal domain). Interacts with MAGI1 (via PDZ 2 and 3 domains) forming a tripartite complex with IGSF5/JAM4. Interacts with DDN; the interaction is direct. Self-associates (via the Ig-like domains). Also interacts (via the Ig-like domains) with KIRREL/NEPH1 and KIRREL2; the interaction with KIRREL is dependent on KIRREL glycosylation. Forms a complex with ACTN4, CASK, IQGAP1, MAGI2, SPTAN1 and SPTBN1 (By similarity). Interacts with NPHS2. |
| 亚细胞定位 | Cell membrane; Single-pass type I membrane protein (Potential). Note=Predominantly located at podocyte slit diaphragm between podocyte foot processes. Also associated with podocyte apical plasma membrane. |
| 组织特异性 | Specifically expressed in podocytes of kidney glomeruli. Expressed in kidney glomeruli. In the embryo,expressed in the mesonephric kidney at E11 with strong expression in cranial tubules with podocyte-like structures. Expression is observed in the podocytes of the developing kidney from E13. High expression is also detected in the developing cerebellum, hindbrain, spinal cord, retina and hypothalamus. Expressed in skeletal muscle during myoblast fusion such as in the adult following acute injury and in the embryo but not detected in uninjured adult skeletal muscle. Isoform 1 and isoform 2 are expressed in the newborn brain and developing cerebellum. Isoform 1 is the predominant isoform in adult kidney. |
| 翻译后修饰 | Phosphorylated at Tyr-1193 by FYN, leading to the recruitment and activation of phospholipase C-gamma-1/PLCG1. DISEASE : Defects in NPHS1 are the cause of nephrotic syndrome type 1 (NPHS1) [MIM:256300]; also known as Finnish congenital nephrosis (CNF). A renal disease characterized clinically by proteinuria, hypoalbuminemia, hyperlipidemia, and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form and progress to end-stage renal failure. |
| 相似性 | Belongs to the immunoglobulin superfamily. Contains 1 fibronectin type-III domain. Contains 8 Ig-like C2-type (immunoglobulin-like) domains. |
| 功能 | Seems to play a role in the development or function of the kidney glomerular filtration barrier. Regulates glomerular vascular permeability. May anchor the podocyte slit diaphragm to the actin cytoskeleton. Plays a role in skeletal muscle formation through regulation of myoblast fusion. |
| 保存条件 | Stored at -70℃ or -20℃. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 背景资料 | This gene encodes a member of the immunoglobulin family of cell adhesion molecules that functions in the glomerular filtration barrier in the kidney. The gene is primarily expressed in renal tissues, and the protein is a type-1 transmembrane protein found at the slit diaphragm of glomerular podocytes. The slit diaphragm is thought to function as an ultrafilter to exclude albumin and other plasma macromolecules in the formation of urine. Mutations in this gene result in Finnish-type congenital nephrosis 1, characterized by severe proteinuria and loss of the slit diaphragm and foot processes.[provided by RefSeq, Oct 2009] |
The purity of the protein is greater than 90% as determined by reducing SDS-PAGE.
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Recombinant human Nephrin, His (bs-41299P)-100ug/500ug/20ug
￥580 - 5680
