Anti-GBA 抗体 [EPR5142] (ab125065)

製品の概要

  • 製品名Anti-GBA antibody [EPR5142]
    GBA 一次抗体 製品一覧
  • 製品の詳細
    Rabbit monoclonal [EPR5142] to GBA
  • アプリケーション適用あり: WB, IHC-Pmore details
    適用なし: Flow Cyt,ICC or IP
  • 種交差性
    交差種: Human
    非交差種: Mouse, Rat
  • 免疫原

    Synthetic peptide, corresponding to residues in Human GBA.

  • ポジティブ・コントロール
    • Human colon tissue; Saos-2, MCF7 and SH-SY5Y cell lysates.
  • 特記事項

    This product is a recombinant rabbit monoclonal antibody.

    Produced using Abcam’s RabMAb® technology. RabMAb® technology is covered by the following U.S. Patents, No. 5,675,063 and/or 7,429,487.

     

    Alternative versions available:

    Anti-GBA antibody (HRP) [EPR5142] (ab200856)

    Anti-GBA antibody (Biotin) [EPR5142] (ab201496)

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab125065 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 1/1000 - 1/10000. Detects a band of approximately 60 kDa (predicted molecular weight: 60 kDa).
IHC-P 1/50 - 1/100. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
  • 追加情報Is unsuitable for Flow Cyt,ICC or IP.
  • ターゲット情報

    • 関連疾患Defects in GBA are the cause of Gaucher disease (GD) [MIM:230800]; also known as glucocerebrosidase deficiency. GD is the most prevalent lysosomal storage disease, characterized by accumulation of glucosylceramide in the reticulo-endothelial system. Different clinical forms are recognized depending on the presence (neuronopathic forms) or absence of central nervous system involvement, severity and age of onset.
      Defects in GBA are the cause of Gaucher disease type 1 (GD1) [MIM:230800]; also known as adult non-neuronopathic Gaucher disease. GD1 is characterized by hepatosplenomegaly with consequent anemia and thrombopenia, and bone involvement. The central nervous system is not involved.
      Defects in GBA are the cause of Gaucher disease type 2 (GD2) [MIM:230900]; also known as acute neuronopathic Gaucher disease. GD2 is the most severe form and is universally progressive and fatal. It manifests soon after birth, with death generally occurring before patients reach two years of age.
      Defects in GBA are the cause of Gaucher disease type 3 (GD3) [MIM:231000]; also known as subacute neuronopathic Gaucher disease. GD3 has central nervous manifestations.
      Defects in GBA are the cause of Gaucher disease type 3C (GD3C) [MIM:231005]; also known as pseudo-Gaucher disease or Gaucher-like disease.
      Defects in GBA are the cause of Gaucher disease perinatal lethal (GDPL) [MIM:608013]. It is a distinct form of Gaucher disease type 2, characterized by fetal onset. Hydrops fetalis, in utero fetal death and neonatal distress are prominent features. When hydrops is absent, neurologic involvement begins in the first week and leads to death within 3 months. Hepatosplenomegaly is a major sign, and is associated with ichthyosis, arthrogryposis, and facial dysmorphism.
      Note=Perinatal lethal Gaucher disease is associated with non-immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders.
      Defects in GBA contribute to susceptibility to Parkinson disease (PARK) [MIM:168600]. A complex neurodegenerative disorder characterized by bradykinesia, resting tremor, muscular rigidity and postural instability. Additional features are characteristic postural abnormalities, dysautonomia, dystonic cramps, and dementia. The pathology of Parkinson disease involves the loss of dopaminergic neurons in the substantia nigra and the presence of Lewy bodies (intraneuronal accumulations of aggregated proteins), in surviving neurons in various areas of the brain. The disease is progressive and usually manifests after the age of 50 years, although early-onset cases (before 50 years) are known. The majority of the cases are sporadic suggesting a multifactorial etiology based on environmental and genetic factors. However, some patients present with a positive family history for the disease. Familial forms of the disease usually begin at earlier ages and are associated with atypical clinical features.
    • 配列類似性Belongs to the glycosyl hydrolase 30 family.
    • 細胞内局在Lysosome membrane. Interaction with saposin-C promotes membrane association.
    • Information by UniProt
    • 参照データベース
    • 別名
      • Acid beta glucosidase antibody
      • Acid beta-glucosidase antibody
      • Alglucerase antibody
      • Beta glucocerebrosidase antibody
      • BETA GLUCOSIDASE, ACID antibody
      • Beta-glucocerebrosidase antibody
      • betaGC antibody
      • D glucosyl N acylsphingosine glucohydrolase antibody
      • D-glucosyl-N-acylsphingosine glucohydrolase antibody
      • EC 3.2.1.45 antibody
      • GBA antibody
      • Gba protein antibody
      • GBA1 antibody
      • GC antibody
      • GCase antibody
      • GCB antibody
      • GLCM_HUMAN antibody
      • GLUC antibody
      • Glucocerebrosidase (alt.) antibody
      • Glucocerebrosidase antibody
      • GLUCOCEREBROSIDASE PSEUDOGENE antibody
      • Glucosidase beta antibody
      • Glucosidase, beta, acid antibody
      • Glucosidase, beta; acid (includes glucosylceramidase) antibody
      • Glucosylceramidase antibody
      • Imiglucerase antibody
      • Lysosomal glucocerebrosidase antibody
      • OTTHUMP00000033992 antibody
      • OTTHUMP00000033993 antibody
      see all

    Anti-GBA antibody [EPR5142] 画像

    • ab125065, at a 1/50 dilution, staining GBA in paraffin-embedded Human colon tissue by immunohistochemistry.
    • All lanes : Anti-GBA antibody [EPR5142] (ab125065) at 1/1000 dilution

      Lane 1 : Saos-2 cell lysate
      Lane 2 : MCF7 cell lysate
      Lane 3 : SH-SY5Y cell lysate

      Lysates/proteins at 10 µg per lane.

      Secondary
      Goat anti-Rabbit HRP at 1/2000 dilution
      Developed using the ECL technique

      Predicted band size : 60 kDa

    Anti-GBA antibody [EPR5142] (ab125065) 使用論文

    ab125065 has not yet been referenced specifically in any publications.

    Product Wall

    Abcam guarantees this product to work in the species/application used in this Abreview.
    Application Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
    Sample Human Tissue sections (Tonsil)
    Antigen retrieval step Heat mediated - Buffer/Enzyme Used: Citric acid
    Permeabilization No
    Specification Tonsil
    Blocking step BSA as blocking agent for 10 minute(s) · Concentration: 1% · Temperature: 21°C
    Fixative Formaldehyde
    Username

    Mr. Carl Hobbs

    Verified customer

    投稿 Dec 08 2015

    Application Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
    Sample Pig Tissue sections (Jejunum)
    Antigen retrieval step Heat mediated - Buffer/Enzyme Used: Citric acid
    Permeabilization No
    Specification Jejunum
    Blocking step BSA as blocking agent for 10 minute(s) · Concentration: 1% · Temperature: 21°C
    Fixative Formaldehyde
    Username

    Mr. Carl Hobbs

    Verified customer

    投稿 Dec 01 2015

    Application Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
    Blocking step BSA as blocking agent for 10 minute(s) · Concentration: 1% · Temperature: 21°C
    Antigen retrieval step Heat mediated - Buffer/Enzyme Used: Citric acid
    Sample Rat Tissue sections (Colon)
    Specification Colon
    Permeabilization No
    Fixative Formaldehyde
    Username

    Mr. Carl Hobbs

    Verified customer

    投稿 Mar 05 2015

    Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"