NDUFB11 Antibody
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中文名稱：NDUFB11兔多克隆抗體
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貨號：CSB-PA015643GA01HU
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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：NDUFB11; UNQ111/PRO1064; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial; Complex I-ESSS; CI-ESSS; NADH-ubiquinone oxidoreductase ESSS subunit; Neuronal protein 17.3; Np17.3; p17.3
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宿主：Rabbit
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反應種屬：Human
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免疫原：Human NDUFB11
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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 0.02% 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：Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.
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基因功能參考文獻：
- recurring mutation, c.276_278del, p.F93del, in NDUFB11, a mitochondrial respiratory complex I-associated protein encoded on the X chromosome, in 5 males with a variably syndromic, normocytic congenital sideroblastic anemia. PMID: 27488349
- This is the third report that describes a mutation in NDUFB11, but all are associated with a different phenotype. Our results further expand the molecular spectrum and associated clinical phenotype of NDUFB11 defects. PMID: 27102574
- The novel NDUFB11 mutation may cause a complex 1 deficiency in synergy with additional unknown mtDNA variants. PMID: 25921236
- Mutations in NDUFB11, encoding a complex I component of the mitochondrial respiratory chain, cause microphthalmia with linear skin defects syndrome. PMID: 25772934
- the post-transcriptional regulation of the Ndufb11 gene can be involved in the programmed cell death process PMID: 23246602
- NDUFB11 did not seem to influence risk and age at onset of visual loss in a total of 65 individuals from 35 Italian Leber hereditary optic neuropathy patients. PMID: 17292333
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相關疾病：Linear skin defects with multiple congenital anomalies 3 (LSDMCA3); Mitochondrial complex I deficiency (MT-C1D)
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亞細胞定位：Mitochondrion inner membrane; Single-pass membrane protein.
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蛋白家族：Complex I NDUFB11 subunit family
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組織特異性：Ubiquitous.
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數據庫鏈接：
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