| 克隆類型 ： |
Rabbit Polyclonal IgG |
|
免 疫 原 ： |
Peptide derived from Hu ACOX1. |
|
純化方式 ： |
Immunogen affinity purified |
|
緩 沖 液 ： |
PBS, pH 7.4(1%BSA and 0.1% Sodium azide) |
|
產品描述 ： |
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. |
| 適用物種 ： |
Hu, Mo, Rat, Pig, Sheep, |
| 應用說明 ： |
WB: 1:200-1:1000 E: 1:500-1:1000 IHC: 1:200-1:1000 IF: 1:100-1:500 |
| 別 名 ： |
ACOX1; ACOX1_Hu; AOX antibody Palmitoyl CoA oxidase; Palmitoyl-CoA oxidase; Peroxisomal acyl coenzyme A oxidase 1; Straight chain acyl CoA oxidase. |
| 保存條件 ： |
Store at -20°C for one year. Avoid repeated freeze-thaw cycles. At 4°C for 2-3 weeks after reconstituted. |
| 標 記 物 ： |
PLLABS公司可供應生物素、辣根過氧化物酶、熒光素標記抗體 |
|
產品說明 ： |
|
|
