製品の概要

  • 製品名Anti-ALAS2 antibody
    ALAS2 一次抗体 製品一覧
  • 製品の詳細
    Mouse monoclonal to ALAS2
  • アプリケーション適用あり: WB, ELISAmore details
  • 種交差性
    交差種: Mouse, Human
    交差が予測される動物種: Horse, Cow, Chimpanzee
  • 免疫原

    Recombinant fragment: MVTAAMLLQC CPVLARGPTS LLGKVVKTHQ FLFGIGRCPI LATQGPNCSQ IHLKATKAGG DSPSWAKGHC PFMLSELQDG KSKIVQKAAP EVQEDVKAFK , corresponding to N terminal amino acids 1 - 101 of Human ALAS2 (NP_000023).

  • ポジティブ・コントロール
    • Raw 264.7 cell lysate

製品の特性

関連製品

アプリケーション

Our Abpromise guarantee covers the use of ab90574 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

アプリケーション Abreviews 特記事項
WB Use a concentration of 1 - 5 µg/ml. Predicted molecular weight: 65 kDa.
ELISA Use at an assay dependent dilution.

ターゲット情報

  • 組織特異性Erythroid specific.
  • パスウェイPorphyrin metabolism; protoporphyrin-IX biosynthesis; 5-aminolevulinate from glycine: step 1/1.
  • 関連疾患Defects in ALAS2 are a cause of anemia sideroblastic X-linked (XLSA) [MIM:300751]. Sideroblastic anemia is characterized by anemia of varying severity, hypochromic peripheral erythrocytes, systemic iron overload secondary to chronic ineffective erythropoiesis, and the presence of bone marrow ringed sideroblasts. Sideroblasts are characterized by iron-loaded mitochondria clustered around the nucleus. XLSA shows a variable hematologic response to pharmacologic doses of pyridoxine.
    Defects in ALAS2 are the cause of erythropoietic protoporphyria X-linked dominant (XLDPT) [MIM:300752]. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. XLDPT is a form of porphyria characterized biochemically by a high proportion of zinc-protoporphyrin in erythrocytes, in which a mismatch between protoporphyrin production and the heme requirement of differentiating erythroid cells leads to overproduction of protoporphyrin in amounts sufficient to cause photosensitivity and liver disease. Note=Gain of function mutations in ALS2 are responsible for XLDPT, but they can also be a possible aggravating factor in congenital erythropoietic porphyria and other erythropoietic disorders caused by mutations in other genes (PubMed:21309041).
  • 配列類似性Belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family.
  • 細胞内局在Mitochondrion matrix.
  • Information by UniProt
  • 参照データベース
  • 別名
    • 5 @aminolevulinate synthase erythroid specific antibody
    • 5 aminolevulinate synthase 2 antibody
    • 5 aminolevulinate synthase 5 aminolevulinate synthase 2 antibody
    • 5 aminolevulinate synthase erythroid specific mitochondrial antibody
    • 5 aminolevulinic acid synthase 2 antibody
    • 5 aminolevulinic acid synthase antibody
    • 5-aminolevulinate synthase antibody
    • 5-aminolevulinic acid synthase 2 antibody
    • Alas 2 antibody
    • ALAS antibody
    • ALAS E antibody
    • ALAS, erythroid antibody
    • ALAS-E antibody
    • Alas2 antibody
    • ALASE antibody
    • Aminolevulinate delta synthase 2 antibody
    • Aminolevulinic acid synthase 2, erythroid antibody
    • ANH1 antibody
    • ASB antibody
    • Delta ALA synthase 2 antibody
    • Delta ALA synthetase antibody
    • Delta aminolevulinate synthase 2 antibody
    • Delta aminolevulinate synthase antibody
    • Delta-ALA synthase 2 antibody
    • Delta-aminolevulinate synthase 2 antibody
    • Erythroid specific ALAS antibody
    • erythroid-specific antibody
    • FLJ93603 antibody
    • HEM0_HUMAN antibody
    • mitochondrial antibody
    • OTTHUMP00000023388 antibody
    • OTTHUMP00000023389 antibody
    • OTTMUSP00000020679 antibody
    • RP23-338A17.1 antibody
    • SIDBA1 antibody
    • XLDPP antibody
    • XLEPP antibody
    • XLSA antibody
    see all

Anti-ALAS2 antibody 画像

  • Anti-ALAS2 antibody (ab90574) at 5 µg/ml + Raw 264.7 cell lysate at 10 µg

    Predicted band size : 65 kDa

Anti-ALAS2 antibody (ab90574) 使用論文

ab90574 has not yet been referenced specifically in any publications.

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