甘油酯激酶线粒体抗体
规格:1尘驳/1尘濒
英文名: Acylglycerol Kinase
别名: mitochondrial; Acylglycerol kinase; Acylglycerol kinase mitochondrial; agk; AGK_HUMAN; hAGK; HsMuLK; MuLK; Multi substrate lipid kinase; Multi-substrate lipid kinase; Multiple substrate lipid kinase.
分子量: 47kDa
储存液:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型:笔辞濒测肠濒辞苍补濒
亚型:滨驳骋
纯化方法:affinity purified by Protein A
**原:KLH conjugated synthetic peptide derived from human Acylglyc
交叉反应:Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Sheep,
细胞定位:细胞浆 细胞膜 线粒体
甘油酯激酶线粒体抗体产物介绍:background: The protein encoded by this gene is a mitochondrial membrane protein involved in lipid and glycerolipid metabolism. The encoded protein is a lipid kinase that catalyzes the formation of phosphatidic and lysophosphatidic acids. Defects in this gene have been associated with mitochondrial DNA depletion syndrome 10. Function: Lipid kinase that can phosphorylate both monoacylglycerol and diacylglycerol to form lysophosphatidic acid (LPA) and phosphatidic acid (PA), respectively. Does not phosphorylate sphingosine. Overexpression increases the formation and secretion of LPA, resulting in transactivation of EGFR and activation of the downstream MAPK signaling pathway, leading to increased cell growth. Subcellular Location: Mitochondrion membrane. Tissue Specificity: Highly expressed in muscle, heart, kidney and brain. DISEASE: Defects in AGK are the cause of mitochondrial DNA depletion syndrome type 10 (MTDPS10) [MIM:212350]. 甘油酯激酶线粒体抗体An autosomal recessive mitochondrial disorder characterized by congenital cataracts, hypertrophic cardiomyopathy, skeletal myopathy, exercise intolerance, and lactic acidosis. Mental development is normal, but affected individuals may die early from cardiomyopathy. Defects in AGK are the cause of cataract, congenital, autosomal recessive type 5 (CATC5) [MIM:614691]. CATC5 consists of an opacification of the crystalline lens of the eye becoming evident at birth. It frequently results in visual impairment or blindness. Opacities vary in morphology, are often 甘油酯激酶线粒体抗体confined to a portion of the lens, and may be static or progressive. In general, the more posteriorly located and dense an opacity, the greater the impact on visual function. Similarity: Contains 1 DAGKc domain. Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产甘油酯激酶线粒体抗体品应用:WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复) not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域:肿瘤 细胞生物 信号转导 激酶和磷酸酶 肿瘤细胞生物标志物 线粒体
储存条件: Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源: Rabbit
外观: Lyophilized or Liquid