LIAS Antibody
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中文名稱：LIAS兔多克隆抗體
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貨號：CSB-PA012927LA01HU
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規格：￥440
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圖片：
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Western blot
All lanes: LIAS antibody at 2µg/ml
Lane 1: MCF-7 whole cell lysate
Lane 2: K562 whole cell lysate
Secondary
Goat polyclonal to rabbit IgG at 1/10000 dilution
Predicted band size: 42, 37, 38 kDa
Observed band size: 42 kDa -
Immunohistochemistry of paraffin-embedded human small intestine tissue using CSB-PA012927LA01HU at dilution of 1:100
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其他：
產品詳情
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產品名稱：Rabbit anti-Homo sapiens (Human) LIAS Polyclonal antibody
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Uniprot No.：
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基因名：LIAS
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別名：LIAS antibody; LAS antibody; HUSSY-01Lipoyl synthase antibody; mitochondrial antibody; EC 2.8.1.8 antibody; Lipoate synthase antibody; LS antibody; Lip-syn antibody; Lipoic acid synthase antibody
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宿主：Rabbit
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反應種屬：Human
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免疫原：Recombinant Human Lipoyl synthase, mitochondrial protein (28-372AA)
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免疫原種屬：Homo sapiens (Human)
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標記方式：Non-conjugated
本頁面中的產品，LIAS Antibody (CSB-PA012927LA01HU)，的標記方式是Non-conjugated。對于LIAS Antibody，我們還提供其他標記。見下表：
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克隆類型：Polyclonal
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抗體亞型：IgG
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純化方式：>95%, Protein G 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：Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 -
產品提供形式：Liquid
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應用范圍：ELISA, WB, IHC
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推薦稀釋比：
Application Recommended Dilution WB 1:500-1:5000 IHC 1:20-1:200 -
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：Catalyzes the radical-mediated insertion of two sulfur atoms into the C-6 and C-8 positions of the octanoyl moiety bound to the lipoyl domains of lipoate-dependent enzymes, thereby converting the octanoylated domains into lipoylated derivatives.
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基因功能參考文獻：
- this study used a bioinformatics approach to predict its structure. . A homology model for LIAS protein was generated using X-ray crystallographic structure of Thermosynechococcus elogatsu. The active site of LIAS protein was mapped and docked with S-Adenosyl Methionine PMID: 27717843
- oxoglutarate dehydrogenase (OGDH) and lipoic acid synthase (LIAS), which when mutated stabilize HIF1alpha in a non-hydroxylated form. PMID: 27923773
- heterozygous mutations (c.738-2A>G and c.929T>C (p.Met310Thr)) in LIAS. PMID: 26108146
- Patients with LIAS nonketotic hyperglycinemia varied in disease severity and cortical involvement. PMID: 24334290
- We identified the homozygous mutation c.746G>A (p.Arg249His) in LIAS in an individual with neonatal-onset epilepsy, muscular hypotonia, lactic acidosis, and elevated glycine concentration in plasma and urine PMID: 22152680
- Lipoic acid synthetase deficiency results in an overall disturbance in the antioxidant defense network, leading to increased inflammation, insulin resistance, and mitochondrial dysfunction. PMID: 19074983
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相關疾?。?/div>Hyperglycinemia, lactic acidosis, and seizures (HGCLAS)亞細胞定位：Mitochondrion.蛋白家族：Radical SAM superfamily, Lipoyl synthase family數據庫鏈接：
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