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OAT Antibody

  • 货号：
    CSB-PA020180
  • 规格：
    ￥880
  • 其他：

产品详情

  • Uniprot No.：
    P04181
  • 基因名：
    OAT
  • 别名：
    OAT antibody; Ornithine aminotransferase antibody; mitochondrial antibody; EC 2.6.1.13 antibody; Ornithine delta-aminotransferase antibody; Ornithine--oxo-acid aminotransferase) [Cleaved into: Ornithine aminotransferase antibody; hepatic form; Ornithine aminotransferase antibody; renal form] antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Synthesized peptide derived from the Internal region of Human OAT.
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 产品提供形式：
    Liquid
  • 应用范围：
    WB, ELISA
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:2000
    ELISA 1:5000
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    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.

产品评价

靶点详情

  • 功能：
    Catalyzes the reversible interconversion of L-ornithine and 2-oxoglutarate to L-glutamate semialdehyde and L-glutamate.
  • 基因功能参考文献：
    1. OAT Val332-to-Met substitution identified in pyridoxine-responsive gyrate atrophy patients does not significantly affect the spectroscopic and kinetic properties of OAT, but during catalysis it makes the protein prone to convert into the apo-form, which undergoes unfolding and aggregation under physiological conditions. PMID: 30251682
    2. identified Arg217 as an important hot-spot at the dimer-dimer interface of hOAT and demonstrated that the artificial dimeric variant R217A exhibits spectroscopic properties, Tm values and catalytic features similar to those of the tetrameric species. This finding indicates that the catalytic unit of hOAT is the dimer. PMID: 28345116
    3. Sequencing of the gene for ornithine aminotransferase reveals a homozygous mutation in our patient (base exchange c.498C>A in Exon 4). PMID: 26259030
    4. Neurogenesis is inhibited by X-OAT during Xenopus embryonic development, but it is essential for Xenopus embryonic development. The Arg 180 and Leu 402 are crucial for these effects of the OAT molecule in development. PMID: 25783604
    5. Our report describes the first case of gyrate atrophy in the Korean population diagnosed by OAT gene analysis and treated with vitamin B6 dietary supplementation. PMID: 24082780
    6. We identified a novel frameshift mutation (p.K169DfsX10) in the OAT gene. While an early arginine-restricted dietary treatment suppressed the fundus changes PMID: 24429551
    7. Data suggest that other factors besides the specific ornithine aminotransferase (OAT) genotype modulate atrophy of choroid and retina (GA) phenotype in patients. PMID: 23076989
    8. OAT was a highly homologous and stable protein located in the mitochondria. PMID: 22989455
    9. Molecular analysis revealed a new deletion c.532_536delTGGGG (p.Trp178X) and a known mutation c.897C>G (p.Tyr299X) in the OAT gene. PMID: 22674428
    10. Fundus autofluorescence imaging can reveal the extent of neurosensory dysfunction in gyrate atrophy patients. PMID: 22182799
    11. analysis of ornithine aminotransferase substrate specificity PMID: 16096275

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  • 相关疾病：
    Hyperornithinemia with gyrate atrophy of choroid and retina (HOGA)
  • 亚细胞定位：
    Mitochondrion matrix.
  • 蛋白家族：
    Class-III pyridoxal-phosphate-dependent aminotransferase family
  • 数据库链接：

    HGNC: 8091

    OMIM: 258870

    KEGG: hsa:4942

    STRING: 9606.ENSP00000357838

    UniGene: Hs.523332