LYRM4 Antibody, HRP conjugated
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中文名稱：LYRM4兔多克隆抗體, HRP偶聯
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貨號：CSB-PA875729LB01HU
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規格：￥880
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其他：
產品詳情
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產品名稱：Rabbit anti-Homo sapiens (Human) LYRM4 Polyclonal antibody
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Uniprot No.：
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基因名：LYRM4
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別名：BC034664 antibody; C6orf149 antibody; CGI 203 antibody; Chromosome 6 open reading frame 149 antibody; Gm903 antibody; Homolog of yeast Isd11 antibody; ISD11 antibody; LYR motif containing 4 antibody; LYR motif-containing protein 4 antibody; LYRM4 antibody; LYRM4_HUMAN antibody; MGC38622 antibody; Mitochondrial matrix Nfs1 interacting protein 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 LYR motif-containing protein 4 protein (1-91AA)
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免疫原種屬：Homo sapiens (Human)
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標記方式：HRP
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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
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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：Required for nuclear and mitochondrial iron-sulfur protein biosynthesis.
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基因功能參考文獻：
- analysis of the NFS1-ISD11-ACP (SDA) complex forms the core of the iron-sulfur (Fe-S) assembly complex and associates with assembly proteins ISCU2, frataxin (FXN), and ferredoxin to synthesize Fe-S clusters PMID: 28634302
- Human Isd11 (ISD11) is a helical protein which exists in solution as an equilibrium between monomer, dimeric and tetrameric species when in the absence of human Nfs1 (NFS1). Recombinant ISD11 expressed in E. coli co-purifies with the bacterial orthologue of NFS1, IscS. PMID: 27427956
- The NFS1/ISD11 complex further interacts with scaffold protein ISCU and regulator protein frataxin, thereby forming a quaternary complex for Fe-S cluster formation. PMID: 28271877
- Molecular dynamics flexible fitting of protein structures docked into the EM map of the model revealed a [FXN(42-210)]24.[NFS1]24.[ISD11]24.[ISCU]24 complex, consistent with the measured 1:1:1:1 stoichiometry of its four components. PMID: 27519411
- Our findings highlight that the ISD11 R68A/R68L mutation display reduced affinity to form a stable subcomplex with NFS1, and thereby fails to prevent NFS1 aggregation resulting in impairment of the Fe-S cluster biogenesis PMID: 26342079
- Homozygous mutation in LYRM4 was reported in two patients with combined oxidative phosphorylation deficiency. PMID: 23814038
- LYRM4 downregulation may be one of the mechanisms involved in inefficient oxidative phosphorylation and oxidative stress, increasingly recognised as contributors to schizophrenia pathogenesis. PMID: 21968932
- Strong candidate gene for mitochondrial disease, based on recessive mutations detected in infantile patients PMID: 22277967
- Frataxin interacts with ISD11 and multiple mitochondrial chaperones. PMID: 17331979
- ISD11 is important in the biogenesis of Fe-S clusters in mammalian cells. PMID: 19454487
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相關疾病：Combined oxidative phosphorylation deficiency 19 (COXPD19)
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亞細胞定位：Mitochondrion. Nucleus.
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蛋白家族：Complex I LYR family
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組織特異性：Reduced mRNA levels in Friedreich ataxia patients.
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數據庫鏈接：
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