SAB4300926
Anti-MT-ND1 antibody produced in rabbit
affinity isolated antibody
别名:
EC 1.6.5.3, MT-ND1, MTND1, NADH dehydrogenase subunit 1, NADH-ubiquinone oxidoreductase chain 1
生物来源
rabbit
质量水平
偶联物
unconjugated
抗体形式
affinity isolated antibody
抗体产品类型
primary antibodies
克隆
polyclonal
表单
buffered aqueous solution
分子量
36 kDa
种属反应性
human
浓度
1.0 mg/mL
技术
immunohistochemistry: 1:50-1:100
western blot: 1:500-1:3000 (Cell Lysate)
同位素/亚型
IgG
登记号
P03886
UniProt登记号
运输
wet ice
储存温度
−20°C
靶向翻译后修饰
unmodified
基因信息
human ... MT-ND1(4535)
相关类别
一般描述
Mitochondrially encoded NADH dehydrogenase 1 (MT-ND1) is a component of mitochondrial membrane respiratory chain nicotinamide adenine dinucleotide (NADH) dehydrogenase (Complex I). It comprises eight transmembrane spanning domains and is a conserved protein placed in the hydrophobic arm of the complex I.
免疫原
Synthesized peptide derived from internal of human MT-ND1.
生化/生理作用
Mitochondrially encoded NADH dehydrogenase 1 (MT-ND1) participates in ubiquinone binding and proton pumping. Mutations in the MT-ND1 gene are implicated in Leber′s hereditary optic neuropathy (LHON), as well as in mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS). Also, some heteroplasmic mutations in the MTND1 gene are associated with an overlap syndrome showing the clinical features associated with LHON and MELAS.
The antibody detects endogenous levels of total MT-ND1 protein.
特点和优势
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外形
Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
免责声明
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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储存分类代码
10 - Combustible liquids
WGK
WGK 1
闪点(°F)
Not applicable
闪点(°C)
Not applicable
法规信息
新产品
Mutations of the mitochondrial ND1 gene as a cause of MELAS.
D M Kirby et al.
Journal of medical genetics, 41(10), 784-789 (2004-10-07)
Chiara La Morgia et al.
BMC neurology, 14, 116-116 (2014-06-03)
An increasing number of mitochondrial DNA (mtDNA) mutations, mainly in complex I genes, have been associated with variably overlapping phenotypes of Leber's hereditary optic neuropathy (LHON), mitochondrial encephalomyopathy with stroke-like episodes (MELAS) and Leigh syndrome (LS). We here describe the
Runyu Guo et al.
Cell, 170(6), 1247-1257 (2017-08-29)
The respiratory megacomplex represents the highest-order assembly of respiratory chain complexes, and it allows mitochondria to respond to energy-requiring conditions. To understand its architecture, we examined the human respiratory chain megacomplex-I2III2IV2 (MCI2III2IV2) with 140 subunits and a subset of associated
Emma L Blakely et al.
European journal of human genetics : EJHG, 13(5), 623-627 (2005-01-20)
Pathogenic point mutations in the mitochondrial MTND1 gene have previously been described in association with two distinct clinical phenotypes -- Leber hereditary optic neuropathy (LHON) and mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS). Here we report the first
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