> Antigen, Antibodies, ELISA, Western Blot > Primary Antibody > Polyclonal Antibodies > TFP1/HADHA AntibodyBrand |
Leading Biology | Catalog Number |
APR10398G |
Product Type |
Polyclonal Antibodies | Field of Research |
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Product Overview |
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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Molecular Weight |
83000 Da
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Cellular Localization |
Antigen Cellular Localization:
Mitochondrion.
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Host |
Rabbit
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Species Reactivity |
Human, Mouse, Rat
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Target |
TFP1
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Isotype |
Rabbit IgG
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Symbol |
HADH
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GeneID |
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UniProt ID |
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Function |
Bifunctional subunit.
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Summary |
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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Form |
Liquid |
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Storage & Stability |
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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Applications |
WB
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Synonyms |
Mitochondrial Trifunctional Protein
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Images |
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Specification |
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Quantity |
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Leading Biology Inc.
2600 Hilltop DR, Building G, B Suite C138
Richmond, CA, 94806
Tel: 1-661-524(LBI)-0262
Email: info@leadingbiology.com
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