全部产品分类
品牌 |
Leading Biology | 货号 |
APR10398G |
产品分类 |
Polyclonal Antibodies | 研究领域 |
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产品概述 |
We constantly strive to ensure we provide our customers with the best antibodies. As a result of this work we offer this antibody in purified format.
We are in the process of updating our datasheets. If you have any questions regarding this update, please feel free to contact our technical support team.
This product is a high quality TFP1/HADHA antibody.
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分子量 |
83000 Da
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细胞定位 |
Antigen Cellular Localization:
Mitochondrion.
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宿主 |
Rabbit
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种属反应性 |
Human, Mouse, Rat
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靶点 |
TFP1
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亚型 |
Rabbit IgG
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通用名 |
HADH
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基因ID |
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UniProt ID |
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功能 |
Bifunctional subunit.
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总结 |
Mitochondrial Trifunctional Protein (TFP) is a multienzyme complex of the beta-oxidation cycle. TFP deficiency is a clinically heterogeneous disorder with phenotypes of different severity. The spectrum of diseases range from severe neonatal/infantile cardiomyopathy and early death to mild chronic progressive sensorimotor poly-neuropathy with episodic rhabdomyolysis. Human TFP is an octomer composed of four alpha-subunits and four beta-subunits. Mutations in either subunits may result in general TFP deficiency with reduced activity of all enzymes.
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形式 |
Liquid
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储存条件 |
Store at +4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.
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应用 |
WB
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别名 |
Mitochondrial Trifunctional Protein
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图像 |
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说明书 |
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数量 |
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