NDUFV1 Antibody
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中文名稱：NDUFV1兔多克隆抗體
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貨號：CSB-PA015668GA01HU
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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：
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別名：CI 51kD antibody; CI-51kD antibody; CI51KD antibody; Complex I 51kD antibody; Complex I-51kD antibody; FLJ59059 antibody; mitochondrial antibody; NADH dehydrogenase (ubiquinone) flavoprotein 1 antibody; NADH dehydrogenase [ubiquinone] flavoprotein 1 antibody; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial antibody; NADH dehydrogenase flavoprotein 1 antibody; NADH ubiquinone oxidoreductase 51 kDa subunit antibody; NADH ubiquinone oxidoreductase antibody; NADH ubiquinone oxidoreductase core subunit V1 antibody; NADH-ubiquinone oxidoreductase 51 kDa subunit antibody; NDUFV 1 antibody; ndufv1 antibody; NDUV1_HUMAN antibody; UQOR1 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 NDUFV1
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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：Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor.
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基因功能參考文獻：
- Mutations in the ND6, NDUFV1 or ACAD9 genes are responsible for the mitochondrial complex I deficiency. PMID: 29348607
- we have used a yeast model system to study the molecular consequences of 16 single amino acid substitutions, classified as pathogenic, in the NDUFV1 subunit of complex I PMID: 26345448
- small number of putative de novo variants were transmitted from BAP parents to their ASD offspring, and evidence emerged for a rare duplication CNV at 11p13.3 harboring two putative developmental/neuropsychiatric susceptibility gene(s), GSTP1 and NDUFV1. PMID: 25432440
- The results affirm that NDUFV1 mutations are causative of the phenotype in two siblings affected by a diffuse leukodystrophy. PMID: 23562761
- observed 2 consanguinous siblings with early-onset encephalopathy, medulla, brainstem and mesencephalon lesions and death before 8 months of age, caused by a complex I deficiency; identified a missense mutation in the NDUFV1 gene; the mutation, p.Arg386His, affects a highly conserved residue PMID: 21696386
- study describes clinical, radiological, biochemical and molecular data of 6 patients with Leigh syndrome with novel mutations in NDUFV1 and NDUFS2; 2 siblings were homozygous for previously undescribed R386C mutation in NDUFV1 PMID: 23266820
- significant negative-correlation between left ventricular end-diastolic dimension and NDUFV1 production in dilated cardiomyopathy PMID: 20930427
- Sp1 was abnormally expressed in schizophrenia and its mRNA alteration pattern paralleled that of NDUFV1 and NDUFV2 in schizophrenic patients. PMID: 17786189
- Mutations in the NDUFV1 gene is linked to a delayed mitochondrial network recovery in OXPHOS disorders. PMID: 20153825
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相關疾病：Leigh syndrome (LS); Mitochondrial complex I deficiency (MT-C1D)
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亞細胞定位：Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
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蛋白家族：Complex I 51 kDa subunit family
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數據庫鏈接：
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