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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

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价格库存

50ul

￥ 1280 1

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100ul

￥ 1980 3

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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.

数据库链接

Entrez Gene : 4541 Human

Omim : 516006 Human

SwissProt : P03923 Human

SwissProt : O79882 Pig

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