NDUFB11 Antibody, FITC conjugated

說明書

中文名稱：

NDUFB11兔多克隆抗體, FITC偶聯
貨號：

CSB-PA868343LC01HU
規格：

￥880
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) NDUFB11 Polyclonal antibody
Uniprot No.：

Q9NX14
基因名：

NDUFB11
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
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial protein (30-88AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

FITC
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
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
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

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.
基因功能參考文獻：
1. 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
2. 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
3. The novel NDUFB11 mutation may cause a complex 1 deficiency in synergy with additional unknown mtDNA variants. PMID: 25921236
4. Mutations in NDUFB11, encoding a complex I component of the mitochondrial respiratory chain, cause microphthalmia with linear skin defects syndrome. PMID: 25772934
5. the post-transcriptional regulation of the Ndufb11 gene can be involved in the programmed cell death process PMID: 23246602
6. 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)
亞細胞定位：

Mitochondrion inner membrane; Single-pass membrane protein.
蛋白家族：

Complex I NDUFB11 subunit family
組織特異性：

Ubiquitous.
數據庫鏈接：

HGNC: 20372

OMIM: 252010

KEGG: hsa:54539

UniGene: Hs.521969

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