ACADL,酰基辅酶A脱氢酶长链抗体

产品编号HZ-10150R
英文名称ACADL
中文名称酰基辅酶A脱氢酶长链抗体
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.
说 明 书0.1ml 0.2ml
研究领域肿瘤 细胞生物 免疫学 转录调节因子 线粒体
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Mouse, Rat,
ACADL,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.
分 子 量44kDa
细胞定位细胞浆 线粒体
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human ACADL
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.
ACADL,酰基辅酶A脱氢酶长链抗体PubMedPubMed
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]. 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 adult form, 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

ACADL,酰基辅酶A脱氢酶长链抗体Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.