Overview
| 别名 | Kelch-like protein 41; Kel-like protein 23; Kelch repeat and BTB domain-containing protein 10; Kelch-related protein 1; Sarcosin |
| 基因名 | KLHL41 |
| UniProt ID | O60662 |
| 反应种属 | Human,Mouse |
| 应用 | WB,IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 2S1-S3-G3 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 68 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | WB-1:1000; 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 | Kelch like family member 41 (KLHL41) is a muscle-specific protein essential for skeletal muscle development and sarcomere integrity. It contains BTB, BACK, and Kelch domains, functioning as a substrate adaptor in cullin 3-based ubiquitin ligase complexes. Unlike many adaptors, KLHL41 primarily acts in a nonproteolytic manner to stabilize the giant sarcomeric protein nebulin, preventing its aggregation and degradation. It promotes myofibril assembly by facilitating the lateral fusion of thin filaments and regulates myoblast proliferation. Clinically, biallelic loss-of-function mutations in KLHL41 cause autosomal recessive nemaline myopathy 9, a severe congenital disorder characterized by muscle weakness, respiratory insufficiency, and the presence of nemaline bodies due to disrupted sarcomeric architecture. |
检测原理