NDUFV1 Antibody

說明書

中文名稱：

NDUFV1兔多克隆抗體
貨號：

CSB-PA015668ESR2HU
規格：

￥440
圖片：
Western blot
All lanes: NDUFV1 antibody at 3.46µg/ml + HepG2 whole cell lysate
Secondary
Goat polyclonal to rabbit IgG at 1/10000 dilution
Predicted band size: 51, 50 kDa
Observed band size: 51 kDa

Immunohistochemistry of paraffin-embedded human brain tissue using CSB-PA015668ESR2HU at dilution of 1:100

Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA015668ESR2HU at dilution of 1:100
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) NDUFV1 Polyclonal antibody
Uniprot No.：

P49821
基因名：

NDUFV1
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
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial protein (1-250AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

Antigen Affinity Purified
濃度：

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

PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
產品提供形式：

Liquid
應用范圍：

ELISA, WB, IHC

推薦稀釋比：

Application	Recommended Dilution
WB	1:1000-1:5000
IHC	1:20-1:200

Protocols：

ELISA Protocol
Western Blotting (WB) 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：

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.
基因功能參考文獻：
1. Mutations in the ND6, NDUFV1 or ACAD9 genes are responsible for the mitochondrial complex I deficiency. PMID: 29348607
2. 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
3. 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
4. The results affirm that NDUFV1 mutations are causative of the phenotype in two siblings affected by a diffuse leukodystrophy. PMID: 23562761
5. 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
6. 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
7. significant negative-correlation between left ventricular end-diastolic dimension and NDUFV1 production in dilated cardiomyopathy PMID: 20930427
8. Sp1 was abnormally expressed in schizophrenia and its mRNA alteration pattern paralleled that of NDUFV1 and NDUFV2 in schizophrenic patients. PMID: 17786189
9. 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)
亞細胞定位：

Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
蛋白家族：

Complex I 51 kDa subunit family
數據庫鏈接：

HGNC: 7716

OMIM: 161015

KEGG: hsa:4723

STRING: 9606.ENSP00000322450

UniGene: Hs.7744

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