ACOX1,过氧化物酶酰基辅酶A氧化酶1抗体

产品编号HZ-5021R
英文名称ACOX1
中文名称过氧化物酶酰基辅酶A氧化酶1抗体
别 名ACOX1; ACOX1_HUMAN; AOX antibody Palmitoyl CoA oxidase; Palmitoyl-CoA oxidase; Peroxisomal acyl coenzyme A oxidase 1; Peroxisomal acyl-coenzyme A oxidase 1; SCOX; Straight chain acyl CoA oxidase; Straight-chain acyl-CoA oxidase.
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 免疫学 转录调节因子
抗体来源Rabbit
 ACOX1,过氧化物酶酰基辅酶A氧化酶1抗体克隆类型Polyclonal
交叉反应Human, Mouse, Rat, Pig, Rabbit, 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74kDa
细胞定位细胞浆
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human ACOX1
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.
 ACOX1,过氧化物酶酰基辅酶A氧化酶1抗体PubMedPubMed
产品介绍background:
Defects in ACOX1 are the cause of adrenoleukodystrophy pseudoneonatal (Pseudo-NALD); also known as peroxisomal acyl-CoA oxidase deficiency. Pseudo-NALD is a peroxisomal single-enzyme disorder. Clinical features include mental retardation, leukodystrophy, seizures, mild hepatomegaly, hearing deficit. Pseudo-NALD is characterized by increased plasma levels of very-long chain fatty cids, due to decreased or absent peroxisome acyl-CoA oxidase activity. Peroxisomes are intact and functioning.

Function:
Catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl-CoAs. Isoform 1 shows highest activity against medium-chain fatty acyl-CoAs and activity decreases with increasing chain length. Isoform 2 is active against a much broader range of substrates and shows activity towards very long-chain acyl-CoAs. Isoform 2 is twice as active as isoform 1 against 16-hydroxy-palmitoyl-CoA and is 25% more active against 1,16-hexadecanodioyl-CoA.

Subcellular Location:
Peroxisome.

Tissue Specificity:
Widely expressed with highest levels of isoform 1 and isoform 2 detected in testis. Isoform 1 is expressed at higher levels than isoform 2 in liver and kidney while isoform 2 levels are higher in brain, lung, muscle, white adipose tissue and testis. Levels are almost equal in heart.

DISEASE:
Defects in ACOX1 are the cause of adrenoleukodystrophy pseudoneonatal (Pseudo-NALD) [MIM:264470]; also known as peroxisomal acyl-CoA oxidase deficiency. Pseudo-NALD is a peroxisomal single-enzyme disorder. Clinical features include mental retardation, leukodystrophy, seizures, mild hepatomegaly, hearing deficit. Pseudo-NALD is characterized by increased plasma levels of very-long chain fatty cids, due to decreased or absent peroxisome acyl-CoA oxidase activity. Peroxisomes are intact and functioning.

 ACOX1,过氧化物酶酰基辅酶A氧化酶1抗体Similarity:
Belongs to the acyl-CoA oxidase family.

Gene ID:
51

Database links:
Entrez Gene: 51 Human
Entrez Gene: 11430 Mouse
Entrez Gene: 50681 Rat
Omim: 609751 Human
SwissProt: Q15067 Human
SwissProt: Q9R0H0 Mouse
SwissProt: P07872 Rat
Unigene: 464137 Human
Unigene: 356689 Mouse
Unigene: 31796 Rat

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.