製品の概要

  • 製品名Anti-Fukutin antibody
    Fukutin 一次抗体 製品一覧
  • 製品の詳細
    Rabbit polyclonal to Fukutin
  • アプリケーション適用あり: WB, IHC-P, ICC/IFmore details
  • 種交差性
    交差種: Human
    交差が予測される動物種: Mouse, Rat
  • 免疫原

    Recombinant Human Fukutin protein.

  • ポジティブ・コントロール
    • Human Skeletal Muscle and Placenta tissue. Whole cell lysates from H1299 and HeLa cells. Fixed HeLa cells.

法規制情報

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab113233 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 1/500 - 1/3000. Predicted molecular weight: 54 kDa.
IHC-P Use a concentration of 10 µg/ml.
ICC/IF 1/100 - 1/200.

ターゲット情報

  • 機能May be a glycosyltransferase which participates in glycosylation of alpha-dystroglycan/DAG1. May interact with and reinforce a large complex encompassing the outside and inside of muscle membranes. Could be involved in brain development.
  • 組織特異性Widely expressed with highest expression in brain, heart, pancreas and skeletal muscle. Expressed at similar levels in control fetal and adult brain, but is much reduced in Fukuyama-type congenital dystrophy (FCMD) brains. Expressed in migrating neurons, including Cajar-Retzius cells and adult cortical neurons, as well as hippocampal pyramidal cells and cerebellar Purkinje cells. No expression observed in the glia limitans, the subpial astrocytes (which contribute to basement membrane formation) or other glial cells. In the FCMD brain, neurons in regions with no dysplasia show fair expression, whereas transcripts are nearly undetectable in the overmigrated dysplastic region.
  • 関連疾患Defects in FKTN are the cause of muscular dystrophy-dystroglycanopathy congenital with brain and eye anomalies type A4 (MDDGA4) [MIM:253800]; also called congenital muscular dystrophy Fukuyama type (FCMD) or Walker-Warburg syndrome FKTN-related. MDDGA4 is an autosomal recessive disorder characterized by congenital muscular dystrophy associated with cobblestone lissencephaly and other brain anomalies. Patients suffer from generalized skeletal muscle weakness and hypotonia from early infancy, mental retardation and seizures. Occasional features include optic atrophy, retinal detachment, cardiomyopathy.
    Defects in FKTN are the cause of muscular dystrophy-dystroglycanopathy congenital without mental retardation type B4 (MDDGB4) [MIM:613152]. An autosomal recessive disorder characterized by congenital muscular dystrophy and evidence of dystroglycanopathy. Features included increased serum creatine kinase, generalized weakness, mild white matter changes on brain MRI in some cases, and absence of mental retardation.
    Defects in FKTN are the cause of muscular dystrophy-dystroglycanopathy limb-girdle type C4 (MDDGC4) [MIM:611588]. MDDGC4 is an autosomal recessive degenerative myopathy characterized by progressive weakness of the pelvic and shoulder girdle muscles and elevated serum creatine kinase. The severity of the disease depends on age at onset which may vary from early to late childhood or even adulthood. MDDGC4 is a novel form of LGMD2 and has no brain involvement and a remarkable clinical response to corticosteroids.
    Defects in FKTN are the cause of cardiomyopathy dilated type 1X (CMD1X) [MIM:611615]; also called dilated cardiomyopathy with mild or no proximal muscle weakness. Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.
  • 配列類似性Belongs to the licD transferase family.
  • 細胞内局在Golgi apparatus membrane.
  • Information by UniProt
  • 参照データベース
  • 別名
    • CMD1X antibody
    • FCMD antibody
    • FCMD gene antibody
    • FKTN antibody
    • FKTN_HUMAN antibody
    • Fukutin antibody
    • Fukuyama type congenital muscular dystrophy protein antibody
    • Fukuyama-type congenital muscular dystrophy protein antibody
    • LGMD2M antibody
    • MDDGA4 antibody
    • MDDGB4 antibody
    • MDDGC4 antibody
    • MGC126857 antibody
    • MGC134944 antibody
    • MGC134945 antibody
    • MGC138243 antibody
    • OTTHUMP00000021841 antibody
    • patient fukutin antibody
    see all

Anti-Fukutin antibody 画像

  • ab113233 at 10 µg/ml staining Fukutin in Formalin-fixed, paraffin-embedded Human skeletal muscle tissue
  • ab113233 at 10 µg/ml staining Fukutin in formalin-fixed, paraffin-embedded Human placenta tissue
  • All lanes : Anti-Fukutin antibody (ab113233) at 1/1000 dilution

    Lane 1 : H1299 whole cell lysate
    Lane 2 : HeLa whole cell lysate

    Lysates/proteins at 30 µg per lane.


    Predicted band size : 54 kDa
  • ab113233 at 1/200 dilution staining Fukutin in methanol-fixed HeLa cells. The lower image is costained with Hoechst 33342.

Anti-Fukutin antibody (ab113233) 使用論文

ab113233 has not yet been referenced specifically in any publications.

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