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NADH复合体6抗体
产品名称:
NADH复合体6抗体
英文名称:
MT-ND6 Rabbit pAb
产品类别:
抗体
产品编号:
YS22462R
产品应用:
WB
保质期:
12个月
[价格]
规格 价格 库存
50ul ¥ 1280 1
100ul ¥ 1980 3

产品详情

 英文名称

MT-ND6 Rabbit pAb
中文名称
NADH复合体6抗体
英文别名
Mitochondrially encoded NADH dehydrogenase 6; NADH-ubiquinone oxidoreductase chain 6; MT ND6; mtND6; NADH dehydrogenase subunit 6(complex I); NADH dehydrogenase subunit 6; NADH ubiquinone oxidoreductase chain 6; NADH Ubiquinone Oxidoreductase subunit ND6; NADH6; ND6; NU6M_HUMAN; NU6M_HUMA
产品应用
WB=1:500-2000

Not yet tested in other applications.
Optimal working dilutions must be determined by the end user.

交叉反应
Mouse, Rat (predicted: Rabbit)
抗体来源
Rabbit
免疫原
KLH conjugated synthetic peptide derived from mouse MT-ND6 : 31-130/172
亚型
IgG
性状
Liquid
纯化方法
affinity purified by Protein A
克隆类型
Polyclonal
理论分子量
19 kDa
浓度
1mg/ml
储存液
0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
研究领域

Cancer > Cancer Metabolism > Metabolic signaling pathway > Integration of energy metabolism

Metabolism > Pathways and Processes > Metabolic signaling pathways > Energy transfer pathways > Integration of energy

Metabolism > Pathways and Processes > Mitochondrial Metabolism > Mitochondrial markers

Metabolism > Pathways and Processes > Mitochondrial Metabolism > Oxidative phosphorylation > Complex I

Neuroscience > Sensory System > Visual system

Signal Transduction > Metabolism > Mitochondrial

亚细胞定位
Mitochondrion membrane; Multi-pass membrane protein (Potential).
疾病
Defects in MT-ND6 are a cause of Leber hereditary optic neuropathy (LHON) [MIM:535000]. LHON is a maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes.
Defects in MT-ND6 are a cause of Leber hereditary optic neuropathy with dystonia (LDYT) [MIM:500001]; also called familial dystonia with visual failure and striatal lucencies. LDYT is part of a spectrum of Leber hereditary optic neuropathy. It is characterized by the association of optic atrophy and central vision loss with dystonia.
Defects in MT-ND6 are a cause of mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes syndrome (MELAS) [MIM:540000]. MELAS is a genetically heterogenious disorder, characterized by episodic vomiting, seizures, and recurrent cerebral insults resembling strokes and causing hemiparesis, hemianopsia, or cortical blindness.
Defects in MT-ND6 are a cause of mitochondrial complex I deficiency (MT-C1D) [MIM:252010]. A disorder of the mitochondrial respiratory chain that causes a wide range of clinical manifestations from lethal neonatal disease to adult-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease.
相似性
Belongs to the complex I subunit 6 family.
功能
Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for 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 (By similarity).
保存条件
Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
背景资料
NADH Dehydrogenase subunit 6 (MTND6) is 1 of the 7 mitochondrial DNA (mtDNA) encoded subunits (MTND1, MTND2, MTND3, MTND4L, MTND4, MTND5, MTND6) included among the approximately 41 polypeptides of respiratory Complex I. Complex I accepts electrons from NADH, transfers them to ubiquinone (Coenzyme Q10), and uses the energy released to pump protons across the mitochondria inner membrane. MTND6 has been proposed to be a component of the iron-protein fragment.
Sample:
Spleen (Mouse) Lysate at 40 ug
Spleen (Rat) Lysate at 40 ug
Primary: Anti-MT-ND6 (22462) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 24 kD

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