NDUFA1 Antibody

說明書

中文名稱：

NDUFA1兔多克隆抗體
貨號：

CSB-PA271484
規格：

￥1100
圖片：
The image on the left is immunohistochemistry of paraffin-embedded Human colon cancer tissue using CSB-PA271484(NDUFA1 Antibody) at dilution 1/30, on the right is treated with fusion protein. (Original magnification: ×200)

The image on the left is immunohistochemistry of paraffin-embedded Human brain tissue using CSB-PA271484(NDUFA1 Antibody) at dilution 1/30, on the right is treated with fusion protein. (Original magnification: ×200)
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

Uniprot No.：

O15239
基因名：

NDUFA1
別名：

NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1; Complex I-MWFE; CI-MWFE; NADH-ubiquinone oxidoreductase MWFE subunit
宿主：

Rabbit
反應種屬：

Human
免疫原：

Fusion protein of Human NDUFA1
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
抗體亞型：

IgG
純化方式：

Antigen affinity purification
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

-20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
產品提供形式：

Liquid
應用范圍：

ELISA,IHC

推薦稀釋比：

Application	Recommended Dilution
ELISA	1:2000-1:5000
IHC	1:50-1:200

Protocols：

ELISA Protocol
Immunohistochemistry (IHC) Protocol
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. Results from a study on gene expression variability markers in early-stage human embryos shows that NDUF1A is a putative expression variability marker for the 3-day, 8-cell embryo stage. PMID: 26288249
2. The gene signature of OPA1, CTSA, NDUFA1, STK10 and PRDX1 was able to identify patients post-implant with a sensitivity of 91% and a specificity of 86% in discrimination between post-implant group and healthy controls. PMID: 27177495
3. Fanconi anemia complementation group A mutants show defective respiration through Complex I, diminished ATP production and metabolic sufferance with an increased AMP/ATP ratio. PMID: 23791750
4. Heterozygous mutation in the X chromosomal NDUFA1 gene in a girl with complex I deficiency PMID: 21596602
5. Mutations in the NDUFA1 gene is linked to a delayed mitochondrial network recovery in OXPHOS disorders. PMID: 20153825
6. Species-specific and mutant MWFE proteins effect on the assembly of a functional mammalian mitochondrial complex I. PMID: 11937507
7. A family-based association study finds that the NDUFA1 gene is unlikely to harbor a major visual loss susceptibility locus for Leber hereditary optic neuropathy. PMID: 12084895
8. Oxidative stress and partial deficiencies of mitochondrial complex I are key factors in the pathogenesis of Parkinson's disease. (REVIEW) PMID: 15038604
9. Suppression of NDUFA1 expression could represent a key pathogenic mechanism in the development of basal cell carcinoma PMID: 15854127
10. Two novel p.Gly8Arg and p.Arg37Ser hemizygous mutations in NDUFA1 were identified in two unrelated male patients presenting with Leigh's syndrome and with myoclonic epilepsy and developmental delay. PMID: 17262856
11. hypothesize that the novel G32R mutation in NDUFA1 is causing complex I deficiency either by itself or in synergy with additional mtDNA variants PMID: 19185523
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相關疾病：

Mitochondrial complex I deficiency (MT-C1D)
亞細胞定位：

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

Complex I NDUFA1 subunit family
組織特異性：

Primarily expressed in heart and skeletal muscle.
數據庫鏈接：

HGNC: 7683

OMIM: 252010

KEGG: hsa:4694

STRING: 9606.ENSP00000360492

UniGene: Hs.534168

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