SLC25A15 Antibody
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中文名稱：SLC25A15兔多克隆抗體
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貨號：CSB-PA021481GA01HU
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規格：￥3,900
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其他：
產品詳情
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Uniprot No.：
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基因名：SLC25A15
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別名：HHH antibody; Mitochondrial ornithine transporter 1 antibody; ORC1 antibody; Ornithine transporter 1 antibody; Ornithine transporter, mitochondrial antibody; ORNT1 antibody; ORNT1_HUMAN antibody; SLC25A15 antibody; Solute carrier family 25 (Mitochondrial carrier, ornithine transporter) member 15 antibody; Solute carrier family 25 member 15 antibody
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宿主：Rabbit
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反應種屬：Human,Mouse,Rat
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免疫原：Human SLC25A15
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免疫原種屬：Homo sapiens (Human)
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抗體亞型：IgG
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純化方式：Antigen Affinity purified
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
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產品提供形式：Liquid
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應用范圍：ELISA,WB,IHC
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Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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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.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
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功能：Ornithine-citrulline antiporter. Connects the cytosolic and the intramitochondrial reactions of the urea cycle by exchanging cytosolic ornithine with matrix citrulline. The stoichiometry is close to 1:1.
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基因功能參考文獻：
- characterized mutations of the proposed substrate binding site in ORC1 and ORC2; demonstrated that the residue at position 179 in the 2 soforms is largely responsible for the difference in their substrate specificity;concluded that Arg-179 is a key residue in the opening of the carrier to the matrix side PMID: 22262851
- Mutation analysis revealed two novel mutations in the ORNT1 gene. PMID: 22465082
- Useful insights for in-depth understanding of the molecular mechanism of the HHH syndrome and developing effective drugs against the disease. PMID: 22292090
- novel mutations in patients with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome PMID: 11668643
- expression, reconstitution, functional characterization, and tissue distribution of two human isoforms PMID: 12807890
- Hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome with evidence of mitochondrial dysfunction due to a novel SLC25A15 (ORNT1) gene mutation in a Palestinian family. PMID: 14759633
- The DeltaF 188 mutant was not incorporated into the membrane to the same extent as wild type, but retained significant residual activity and lost stereospecificity. PMID: 16256388
- A novel R275X mutation of the SLC25A15 gene in a Japanese patient with the HHH syndrome PMID: 16376511
- The three patients were homozygous for a novel mutation in ORNT1 with a Gly220Arg change. We suggest including HHH syndrome in the differential diagnosis of patients found to have stroke-like lesions on brain MRI. PMID: 17825324
- Clinical presentations and outcomes varied significantly in HHH syndrome patients homozygous for delF188 mutations in SLC25A15. PMID: 18978333
- 16 additional Hyperornithinemia-hyperammonemia-homocitrullinuria cases were collected and the spectrum of SLC25A15/ORC1 mutations, was expanded. PMID: 19242930
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相關疾病：Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome (HHH syndrome)
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亞細胞定位：Mitochondrion inner membrane; Multi-pass membrane protein.
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蛋白家族：Mitochondrial carrier (TC 2.A.29) family
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組織特異性：Highly expressed in liver, pancreas, testis, lung and small intestine. Lower levels are detected in spleen, kidney, brain and heart.
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數據庫鏈接：
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