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ACSL4 Rabbit pAb (bs-13129R)  
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50ul/1180.00元
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產品編號 bs-13129R
英文名稱 ACSL4 Rabbit pAb
中文名稱 酰基輔酶A合成酶4抗體
別    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;  
研究領域 細胞生物  發育生物學  神經生物學  信號轉導  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 (predicted: Human,Mouse,Rat,Rabbit,Pig,Sheep,Cow,Chicken,Horse)
產品應用 ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 79 kDa
檢測分子量
細胞定位 細胞漿 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ACSL4: 321-420/711 
亞    型
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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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