徐州威格顿生物科技有限公司
威格顿生物
Weigedun Biotechnology
KDSR Mouse mAb
规格信息
| 规格 | 货号 |
|---|---|
| 50ul | WGD-Z-2611512 |
| 100ul | WGD-Z-2611512 |
详细参数
| 别名 | 3-ketodihydrosphingosine reductase; KDS reductase; 3-dehydrosphinganine reductase; Follicular variant translocation protein 1; FVT-1; Short chain dehydrogenase/reductase family 35C member 1 |
| 基因名 | KDSR |
| UniProt ID | Q06136 |
| 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 | 6P4-D5-W3 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 36 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | IHC-1:1000 |
| 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 | 3-ketodihydrosphingosine reductase (KDSR) is an endoplasmic reticulum membrane oxidoreductase that catalyzes the reduction of 3-ketodihydrosphingosine to dihydrosphingosine, representing the second committed step in de novo sphingolipid and ceramide biosynthesis. The enzyme is essential for maintaining ER lipid composition and unfolded protein response (UPR) homeostasis; its loss leads to ER stress and apoptosis. Clinically, biallelic loss-of-function mutations in KDSR cause a syndromic phenotype characterized by thrombocytopenia and progressive keratinization disorders, such as erythrokeratodermia variabilis et progressiva (EKVP4). Additionally, chromosomal rearrangements involving the KDSR gene are recurrent in follicular lymphoma and type II chronic lymphatic leukemia, while altered expression is implicated in T-cell malignancies and hepatic injury. These associations underscore the critical role of KDSR in both systemic lipid metabolism and oncogenesis. |