酰基辅酶A脱氢酶长链抗体
规格：1mg/1ml
英文名： ACADL
别名： mitochondrial; ACAD4; ACADL; ACADL_HUMAN; Acyl Coenzyme A dehydrogenase long chain; FLJ94052; LCAD; Long chain acyl CoA dehydrogenase; Long-chain specific acyl-CoA dehydrogenase.
分子量： 44kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human ACADL
交叉反应：Human, Mouse, Rat,
细胞定位：细胞浆 线粒体
酰基辅酶A脱氢酶长链抗体产品介绍：background: The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family, which is a family of mitochondrial flavoenzymes involved in fatty acid and branched chain amino-acid metabolism. This protein is one of the four enzymes that catalyze the initial step of mitochondrial beta-oxidation of straight-chain fatty acid. Defects in this gene are the cause of long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycemia. [provided by RefSeq]. Subunit: Homotetramer. Subcellular Location: Mitochondrion matrix. DISEASE: Defects in ACADL are a cause of acyl-CoA dehydrogenase very long-chain deficiency (ACADVLD) [MIM:201475]. 酰基辅酶A脱氢酶长链抗体An inborn error of mitochondrial fatty acid beta-oxidation which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form characterized by early onset, high mortality and high incidence of cardiomyopathy; a milder childhood form with later onset, characterized by hypoketotic hypoglycemia, low mortality and rare cardiomyopathy; an ***** form, 酰基辅酶A脱氢酶长链抗体with isolated skeletal muscle involvement, rhabdomyolysis and myoglobinuria, usually triggered by exercise or fasting. Similarity: Belongs to the acyl-CoA dehydrogenase family. Database links: Entrez Gene: 33 Human Entrez Gene: 614508 Cow Entrez Gene: 11363 Mouse Entrez Gene: 396931 Pig Entrez Gene: 25287 Rat Omim: 609576 Human SwissProt: P28330 Human SwissProt: P51174 Mouse SwissProt: P79274 Pig SwissProt: P15650 Rat Unigene: 471277 Human Unigene: 2445 Mouse Unigene: 174 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
酰基辅酶A脱氢酶长链抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=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 **学 转录调节因子 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