Acylglycerol Kinase,甘油酯激酶线粒体抗体

产品编号HZ-6276R
英文名称Acylglycerol Kinase
中文名称甘油酯激酶线粒体抗体
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.
规格价格0.1ml 0.2ml 
说 明 书0.1ml 0.2ml
研究领域肿瘤 细胞生物 信号转导 激酶和***酸酶 肿瘤细胞生物标志物 线粒体
抗体来源Rabbit
克隆类型Polyclonal
Acylglycerol Kinase,甘油酯激酶线粒体抗体交叉反应Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Sheep,
产品应用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.
分 子 量47kDa
细胞定位细胞浆 细胞膜 线粒体
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human Acylglycerol Kinase
Acylglycerol Kinase,甘油酯激酶线粒体抗体亚 型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
PubMedPubMed
产品介绍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.

Acylglycerol Kinase,甘油酯激酶线粒体抗体DISEASE:
Defects in AGK are the cause of mitochondrial DNA depletion syndrome type 10 (MTDPS10) [MIM:212350]. 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 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.