產品編號 | bs-42132P |
英文名稱 | Recombinant mouse ACOX1 protein, C-His |
中文名稱 | 重組小鼠過氧化物酶酰基輔酶A氧化酶1蛋白 |
別 名 | ACOX1; ACOX1_HUMAN; ACOX1_MOUSE; 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. |
理論分子量 | 31.8kDa |
性 狀 | Lyophilized or Liquid |
濃 度 | >0.5mg/ml |
物 種 | Mouse |
序 列 | 386-661/661 |
純 度 | >90% as determined by SDS-PAGE |
表達系統(tǒng) | E.coli |
活性 | Not tested |
標簽 | C-His |
緩 沖 液 | 20mM Tris-Hcl (pH=8.0)with 8M Urea |
保存條件 | Stored at -70℃ or -20℃. 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. |
產品介紹 | 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. SWISS: Q9R0H0 Gene ID: 11430 |
產品圖片 | |
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