全部產(chǎn)品分類
品牌 |
Leading Biology | 貨號 |
APR10398G |
產(chǎn)品分類 |
Polyclonal Antibodies | 研究領(lǐng)域 |
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產(chǎn)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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細(xì)胞定位 |
Antigen Cellular Localization:
Mitochondrion.
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宿主 |
Rabbit
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種屬反應(yīng)性 |
Human, Mouse, Rat
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靶點(diǎn) |
TFP1
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亞型 |
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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總結(jié) |
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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儲存條件 |
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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應(yīng)用 |
WB
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別名 |
Mitochondrial Trifunctional Protein
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圖像 |
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說明書 |
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數(shù)量 |
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