MS702 Mitochondrial Trifunctional Protein
(TFP) Monoclonal Antibody
$295.00 (100 µg)
Please contact us for volume discounts.
Brief Description & Uses
100 ug of monoclonal antibody reactive against the mitochondrial
trifunctional protein (TFP). TFP is part of the mitochondrial fatty
acid beta-oxidation pathway. It is located in the inner mitochondrial
membrane and it catalyzes three out of the four steps in the
beta-oxidation cycle with a specific affinity for long chain
substrates. Inherited deficiency of TFP is a recessive genetic disease
which occurs in approximately 1:38,000 newborns in the US. Deficiency
of LCHAD (the dehydrogenase part of TFP) has also been found in
children of women who develop HELLP syndrome (1:1,000 pregnancies) and
AFLP syndrome (1:13,000 pregnancies), both of which are
life-threatening obstetric conditions. Furthermore, heterozygous
individuals for TFP defects are more susceptible to nonalcoholic
hepatic steatosis, or nonalcoholic fatty liver disease (NAFLD), the
most common form of liver disease in the US with a recorded prevalence
as high as 39%.
Specifications
Product:
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Mitochondrial Trifunctional Protein
(TFP) monoclonal antibody
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Catalog #:
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MS702
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Lot #:
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Clone ID:
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2C2AA5
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Host Species and Isotype:
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Mouse IgG2b
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Immunogen:
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Human Heart Mitochondria
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Concentration:
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1 mg/ml in HEPES-Buffered Saline (HBS) with 0.02% azide as a preservative.
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Purity:
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Near homogeneity as judged by SDS-PAGE. The antibodies were produced in vitro using hybridomas grown in serum-free medium, and then purified by biochemical fractionation.
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Suggested Working Concentration:
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0.5 µg/mL for Western blotting
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Applications:
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Western blotting, immunocapture
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Antigen Species Cross-Reactivity:
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human
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Storage:
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4°C
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Country of Origin:
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USA
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Product References
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References for Trifunctional Protein
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Images
A Western blot image of MS702 with human heart mitochondria (HHM), and human liver mitochondria (HLM). An anti-Complex II 30kDa subunit antibody has been used as a control. MS702 also immunocaptures both the alpha and beta subunits of TFP.
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