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HOGA1 Antibody, FITC conjugated

  • 中文名稱(chēng):
    HOGA1兔多克隆抗體, FITC偶聯(lián)
  • 貨號(hào):
    CSB-PA771464LC01HU
  • 規(guī)格:
    ¥880
  • 其他:

產(chǎn)品詳情

  • 產(chǎn)品名稱(chēng):
    Rabbit anti-Homo sapiens (Human) HOGA1 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    HOGA1
  • 別名:
    HOGA1 antibody; C10orf65 antibody; DHDPSL antibody; 4-hydroxy-2-oxoglutarate aldolase antibody; mitochondrial antibody; EC 4.1.3.16 antibody; Dihydrodipicolinate synthase-like antibody; DHDPS-like protein antibody; Probable 2-keto-4-hydroxyglutarate aldolase antibody; Probable KHG-aldolase antibody; Protein 569272 antibody
  • 宿主:
    Rabbit
  • 反應(yīng)種屬:
    Human
  • 免疫原:
    Recombinant Human 4-hydroxy-2-oxoglutarate aldolase, mitochondrial protein (1-164AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標(biāo)記方式:
    FITC
  • 克隆類(lèi)型:
    Polyclonal
  • 抗體亞型:
    IgG
  • 純化方式:
    >95%, Protein G purified
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 產(chǎn)品提供形式:
    Liquid
  • 儲(chǔ)存條件:
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期:
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
  • 用途:
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產(chǎn)品評(píng)價(jià)

靶點(diǎn)詳情

  • 功能:
    Catalyzes the final step in the metabolic pathway of hydroxyproline.
  • 基因功能參考文獻(xiàn):
    1. Results show that HOGA1 harboring mutations found in primary hyperoxaluria is thermally unstable and targeted for proteolytic degradation leading to an absolute loss of function. PMID: 27096395
    2. Among the seven patients identified with HOGA1 mutations the median onset of clinical symptoms was 1.8 years. Five patients initially presented with urolithiasis, and two other patients presented with urinary tract infection. PMID: 25972204
    3. Our results strongly suggest HOGA1 as a major cause of PH, indicate a greater genetic heterogeneity of hyperoxaluria, and point to a favorable outcome of type III in the context of PH despite incomplete or absent biochemical remission PMID: 22781098
    4. seven different mutations were identified in primary hyperoxaluria type 3 PMID: 22391140
    5. Two crystal forms of DHDPSL were obtained, both of which diffracted X-rays to approximately 2.0 A resolution PMID: 22232173
    6. hHOGA utilizes a type I aldolase reaction mechanism, but employs novel residue interactions for substrate binding PMID: 21998747
    7. Detection of HOGA1 variants in idiopathic calcium oxalate urolithiasis also suggests HOGA1 may be a predisposing factor for this condition PMID: 21896830
    8. DHDPSL is the gene encoding 4-hydroxy-2-oxoglutarate aldolase, catalyzing the final step in the metabolic pathway of hydroxyproline. PMID: 20797690

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  • 相關(guān)疾病:
    Hyperoxaluria primary 3 (HP3)
  • 亞細(xì)胞定位:
    Mitochondrion.
  • 蛋白家族:
    DapA family
  • 數(shù)據(jù)庫(kù)鏈接:

    HGNC: 25155

    OMIM: 613597

    KEGG: hsa:112817

    STRING: 9606.ENSP00000359680

    UniGene: Hs.180346



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