过氧化物酶酰基辅酶A氧化酶1抗体
规格：1mg/1ml
英文名： ACOX1
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; Stra
分子量： 74kDa
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 ACOX1
交叉反应：Human, Mouse, Rat, Pig, Rabbit, Sheep,
细胞定位：细胞浆
过氧化物酶酰基辅酶A氧化酶1抗体产品介绍：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. 过氧化物酶酰基辅酶A氧化酶1抗体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 过氧化物酶酰基辅酶A氧化酶1抗体of very-long chain fatty cids, due to decreased or absent peroxisome acyl-CoA oxidase activity. Peroxisomes are intact and functioning. 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.
过氧化物酶酰基辅酶A氧化酶1抗体产品应用：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.
研究领域：肿瘤 细胞生物 **学 转录调节因子
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid