MT-ND5 Antibody, HRP conjugated

說明書

中文名稱：

MT-ND5兔多克隆抗體, HRP偶聯(lián)
貨號(hào)：

CSB-PA015081LB01HU
規(guī)格：

￥880
其他：

產(chǎn)品詳情
產(chǎn)品評(píng)價(jià)
相關(guān)產(chǎn)品
靶點(diǎn)詳情

產(chǎn)品詳情

產(chǎn)品名稱：

Rabbit anti-Homo sapiens (Human) MT-ND5 Polyclonal antibody
Uniprot No.：

P03915
基因名：

MT-ND5
別名：

Complex I, subunit ND5 antibody; EC 1.6.5.3 antibody; Mitochondrially encoded NADH dehydrogenase 5 antibody; MT ND5 antibody; MT-ND5 antibody; MTND 5 antibody; MTND5 antibody; NAD5 antibody; NADH dehydrogenase subunit 5 (complex I) antibody; NADH dehydrogenase subunit 5 antibody; NADH ubiquinone oxidoreductase , subunit ND5 antibody; NADH ubiquinone oxidoreductase chain 5 antibody; NADH-ubiquinone oxidoreductase chain 5 antibody; NADH5 antibody; ND5 antibody; NU5M_HUMAN antibody
宿主：

Rabbit
反應(yīng)種屬：

Human
免疫原：

Recombinant Human NADH-ubiquinone oxidoreductase chain 5 protein (503-581AA)
免疫原種屬：

Homo sapiens (Human)
標(biāo)記方式：

HRP
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(chǎn)品提供形式：

Liquid
應(yīng)用范圍：

ELISA
Protocols：

ELISA Protocol
儲(chǔ)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(chǎn)品評(píng)價(jià)

靶點(diǎn)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. Essential for the catalytic activity and assembly of complex I.
基因功能參考文獻(xiàn)：
1. MT-ND5 mutation exhibits highly variable neurological manifestations. PMID: 29506874
2. Data suggest that mutations in MT-CO2 and MT-ND5 can be involved in MIDD (maternally inherited diabetes and deafness); a Tunisian family (mother, daughter, son) with clinical features of MIDD associated with retinopathy exhibit mutations in MT-CO2 (m.8241T>G - p. F219C) and MT-ND5 (m.13276G>A - p. M314V); these two mutations could explain retinopathy in some family members. (MT-CO2 = cytochrome c oxidase subunit II) PMID: 27422531
3. A MELAS/Leigh syndrome phenotype caused by a mtDNA mutation [m.13513G>A; p.Asp393Asn] in the ND5 gene encoding the ND5 subunit of respiratory chain complex I was identified in a patient in a second family PMID: 27344355
4. We describe a unique presentation Leber hereditary optic neuropathy (LHON) and mitochondrial encephalopathy, myopathy, lactic acidosis and stroke-like episodes (MELAS) overlap syndrome resulting from a m.13046T>C mutation in a 12-year-old girl. PMID: 26894521
5. FASTKD4 is required to promote expression of ND5. FASTKD4 may promote processing of ND5-CYB precursor or it may stabilize multiple mature RNAs including ND5. PMID: 28335001
6. The m.13565C>T mutation in MTND5 causes defects in both mitochondrial oxidative metabolism and mitochondrial calcium sequestration in a MELAS syndrome fibroblasts. PMID: 27110715
7. Mitochondrially encoded NADH dehydrogenase subunit, complex I extracts energy from NADH, produced by the oxidation of sugars and fats, and traps the energy in a potential difference or voltage across the mitochondrial inner membrane. PMID: 25756807
8. Study demonstrates a link between p53 and Bcl-2 proteins as regulators of ROS production and cellular invasiveness, and reveals complex-I, especially ND5, as their functional target in lung tumor cells. PMID: 25115399
9. Mitochondrial ND5 12338T>C variant is associated with maternally inherited hypertrophic cardiomyopathy in a Chinese pedigree PMID: 22759514
10. Mitochondrial DNA mutations in respiratory complex-I in never-smoker lung cancer patients contribute to lung cancer progression in association with EGFR gene mutation. PMID: 21830212
11. Several lines of evidence suggest that the mitochondrial ND5T12338C mutation is associated with Leber's hereditary optic neuropathy (LHON) in Chinese families. PMID: 21131053
12. ND4 G11696A and ND5 T12338C mutation is likely associated with Leber's hereditary optic neuropathy in two Chinese families PMID: 21482521
13. Patients with ND5*13513 G to A mutation may have a characteristic clinical course and ND5 *13513 G to A might be a preferential candidate mutation of Leigh syndrome. PMID: 21154318
14. One proband had a non-synonymous A14062G mutation in the ND5 gene. PMID: 12031626
15. This study conclude that the G13513A mutation causes a complex I defect when present at unusually low mutant load and may act dominantly. PMID: 14520659
16. A 13513G->A transition in ND5 was identified in a 4-year-old Italian boy atypical Leigh syndrome patient PMID: 14557590
17. The expression of MTND5 was studied in blood platelets during aging. There was increased genetic transcription. PMID: 14759509
18. Heteroplasmic mutations largely segregate PD from controls and may be of major pathogenic importance in idiopathic Parkinson disease. PMID: 15596151
19. In cholera, ND5 is upregulated in the mucosa of the small intestine, which correlates with the virulence of Vibrio cholerae. PMID: 15946665
20. we describe the clinical and genetic characterization of a new LHON mtDNA mutation. The 12848T mutation alters a highly conserved amino acid in the ND5 complex I gene. PMID: 16240359
21. Secondary structure analysis of the ND5 protein further supported the deleterious role of the 12706C mutation PMID: 17317336
22. Complete screening of the mitochondrial genome of 116 patients detected 4 families with an ND5 mutation (including a new m.13511AT mutation), accounting for 27% of the total number of mtDNA gene mutations. PMID: 17400793
23. MT-ND5 gene variation is significantly associated with brain mitochondrial respiratory function in Tibet chicken embryos under hypoxia. PMID: 17614984
24. In contrast to results reported for PD frontal cortex, low-level ND5 mutations between codons 120 and 150 do not accumulate severely in biochemically affected skeletal muscle samples of Parkinson's patients. PMID: 17702497
25. ND5 gene is implicated in mitochondrial dysfunction in schizophrenia. PMID: 17898419
26. Mitochondrial ND5 gene variation associated with encephalomyopathy and mitochondrial ATP consumption PMID: 17940288
27. identified the nt13708A variant as a susceptibility allele to multiple sclerosis PMID: 18270557
28. The G13513A mutation is a common cause of MELAS and LS, even in the absence of obvious maternal inheritance, pathological findings in muscle, or severe complex I deficiency. PMID: 18332249
29. We describe a patient with isolated exercise intolerance caused by a new, maternally inherited mutation in heteroplasmic T>C transition at position 13271 in MTND5. PMID: 18396045
30. 13513G>A mutation in the ND5 is associated with Leigh or Leigh-like disease. PMID: 18495510
31. We suggest that mtDNA G13513A mutation is an important factor in patients with Leigh syndrome associated with WPW syndrome and/or optic atrophy. PMID: 19054921
32. article describes 2 mitochondrial DNA mutations in the ND3 and ND5 genes in patients showing clinical features of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes PMID: 19617458
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相關(guān)疾病：

Leber hereditary optic neuropathy (LHON); Leigh syndrome (LS); Mitochondrial complex I deficiency (MT-C1D); Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes syndrome (MELAS)
亞細(xì)胞定位：

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

Complex I subunit 5 family
數(shù)據(jù)庫鏈接：

HGNC: 7461

OMIM: 252010

KEGG: hsa:4540

STRING: 9606.ENSP00000354813

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