Anti-NU4LM antibody
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概述
- 产品描述catalytic activity:NADH + ubiquinone = NAD(+) + ubiquinol. disease:Defects in MT-ND4 are a cause of Leber hereditary optic neuropathy (LHON). 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. disease:Defects in MT-ND4 are a cause of Leber hereditary optic neuropathy with dystonia (LDYT); 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. disease:Defects in MT-ND4 are a cause of mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes syndrome (MELAS). MELAS is a genetically heterogenious disorder, characterized by episodic vomiting, seizures, and recurrent cerebral insults resembling strokes and causing hemiparesis, hemianopsia, or cortical blindness. disease:Defects in MT-ND4L are a cause of Leber hereditary optic neuropathy (LHON). 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. 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. Belongs to the complex I subunit 4 family. Belongs to the complex I subunit 4L family.
- 产品名称Anti-NU4LM antibody
- 分子量10 kDa
- 种属反应性Human
- 验证应用WB
- 抗体类型兔多抗
- 免疫原Synthesized peptide derived from human protein . at AA range: 10-90
- 偶联Non-conjugated
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性能
- 形态Liquid
- 浓度1 mg/mL.
- 存放说明Store at +4℃ after thawing. Aliquot store at -20℃ or -80℃. Avoid repeated freeze / thaw cycles.
- 存储缓冲液Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.
- 亚型IgG
- 纯化方式Immunogen affinity purified.
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特别提示：本公司的所有产品仅可用于科研实验，严禁用于临床医疗及其他非科研用途！
