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RabbitAnti-ACSL4 Rabbit pAb  antibody (bs-13129R)
~~~促销,代码KXJ230206~~~
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价
产品编号 bs-13129R
英文名称 ACSL4 Rabbit pAb
中文名称 酰基辅酶A合成酶4抗体
别    名 ACSL4_HUMAN; Long-chain-fatty-acid--CoA ligase 4; ACS4; FACL4; LACS4; EC:6.2.1.3; Arachidonate--CoA ligase; Long-chain acyl-CoA synthetase 4(LACS 4); acyl-CoA synthetase long chain family member 4; MRX63; MRX68; XLID63;  
研究领域 细胞生物  发育生物学  神经生物学  信号转导  新陈代谢  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, Chicken, Pig, Cow, Horse, Rabbit, Sheep, )
产品应用 ELISA=1:5000-10000 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 79 kDa
检测分子量
细胞定位 细胞浆 细胞膜 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ACSL4: 321-420/711 
亚    型
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 The protein encoded by this gene is an isozyme of the long-chain fatty-acid-coenzyme A ligase family. Although differing in substrate specificity, subcellular localization, and tissue distribution, all isozymes of this family convert free long-chain fatty acids into fatty acyl-CoA esters, and thereby play a key role in lipid biosynthesis and fatty acid degradation. This isozyme preferentially utilizes arachidonate as substrate. The absence of this enzyme may contribute to the cognitive disability or Alport syndrome. Alternative splicing of this gene generates multiple transcript variants. [provided by RefSeq, Jan 2016]

Function:
Activation of long-chain fatty acids for both synthesis of cellular lipids, and degradation via beta-oxidation. Preferentially uses arachidonate and eicosapentaenoate as substrates.

Subcellular Location:
Mitochondrion outer membrane. Peroxisome membrane. Microsome membrane. Endoplasmic reticulum membrane.

DISEASE:
Defects in ACSL4 are the cause of mental retardation X-linked type 63 (MRX63) [MIM:300387]. Mental retardation is a mental disorder characterized by significantly sub-average general intellectual functioning associated with impairments in adaptative behavior and manifested during the developmental period. Non-syndromic mental retardation patients do not manifest other clinical signs.
Defects in ACSL4 are involved in Alport syndrome with mental retardation midface hypoplasia and elliptocytosis (ATS-MR) [MIM:300194]. A X-linked contiguous gene deletion syndrome characterized by glomerulonephritis, deafness, mental retardation, midface hypoplasia and elliptocytosis.

Similarity:
Belongs to the ATP-dependent AMP-binding enzyme family.

SWISS:
O60488

Gene ID:
2182

Database links:

Entrez Gene: 2182 Human

Entrez Gene: 50790 Mouse

Entrez Gene: 113976 Rat

Omim: 300157 Human

SwissProt: O60488 Human

SwissProt: Q9QUJ7 Mouse

SwissProt: O35547 Rat

Unigene: 268785 Human

Unigene: 391337 Mouse

Unigene: 87821 Rat



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