徐州威格顿生物科技有限公司
威格顿生物
Weigedun Biotechnology
MRAP Mouse mAb
规格信息
| 规格 | 货号 |
|---|---|
| 50ul | WGD-Z-2611840 |
| 100ul | WGD-Z-2611840 |
详细参数
| 别名 | Melanocortin-2 receptor accessory protein; B27; Fat cell-specific low molecular weight protein; Fat tissue-specific low MW protein |
| 基因名 | MRAP |
| UniProt ID | Q8TCY5 |
| 反应种属 | Human |
| 应用 | IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG2a |
| 克隆号 | 1M5-M7-P9 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 19 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | IHC-1:200-1:250 |
| 存储缓冲液 | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.09% sodium azide |
| 保存温度 | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| 背景信息 | Melanocortin 2 receptor accessory protein (MRAP) is a small, single-pass transmembrane protein that forms antiparallel homodimers with unique dual topology. It is essential for the trafficking and functional maturation of the melanocortin 2 receptor (MC2R), also known as the ACTH receptor. MRAP facilitates the exit of MC2R from the endoplasmic reticulum, ensures its correct glycosylation, and enables its signaling at the plasma membrane. While primarily required for MC2R, MRAP also modulates the ligand sensitivity of other melanocortin receptors (MC1R, MC3R, MC4R, and MC5R). Clinically, biallelic loss-of-function mutations in MRAP lead to familial glucocorticoid deficiency type 2. This condition is characterized by severe cortisol deficiency and ACTH resistance, often presenting in infancy with life-threatening hypoglycemia and adrenal crises. |