Mitochondrial trifunctional protein (TFP) subunit alpha (HADHA)
monoclonal antibody
Catalog No. MS702
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$325.00 - 100 µg
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UniProt Number:
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P40939
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Alternate Names:
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Trifunctional enzyme subunit alpha, TP-alpha, 78 kDa gastrin-binding protein, HADH
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Structure and Function:
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The Mitochondrial trifunctional protein (TFP) alpha subunit (HADHA) is a 79 kDa enzyme which has two enzymatic functions, the hydration of enoyl-CoA and the dehydrogenation of 3-hydroxyacyl CoA.
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Disease Associations:
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Defects in HADHA are a cause of trifunctional protein deficiency (TFP deficiency) [MIM:609015], long-chain 3-hydroxyl-CoA dehydrogenase deficiency (LCHAD deficiency) [MIM:609016], and maternal acute fatty liver of pregnancy (AFLP) [MIM:609016].
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Product Specifications
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Applications:
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Western blotting, Immunocytochemistry, Immunoprecipitation, In-Cell ELISA, Flow Cytometry
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Species Reactivity:
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human
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Host Species:
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mouse
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Isotype:
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IgG2b, κ
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Clone ID:
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2C2AA5
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Concentration:
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1.0 mg/mL in Hepes-Buffered Saline (HBS) with 0.02% azide as a preservative.
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Suggested Working Concentration:
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0.5 µg/mL for Western blotting
4 µg/mL for Immunocytochemistry
1 µg/mL for In-Cell ELISA (0.1 µg/well)
1 µg/mL for Flow Cytometry
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Storage Conditions:
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Store at 4°C. Do not freeze.
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Country of Origin:
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USA
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ICC Images
(click to enlarge)
Figure 1. Immunocytochemistry image of MS702 stained human HDFn cells. The cells were paraformaldehyde fixed (4%, 20 min) and Triton X-100 permeabilized (0.1%, 15min). The cells were incubated with the antibody (2C2AA5, 4 µg/ml) for 2h at room temperature or over night at 4°C. The secondary antibody was (green) AlexaFluor® 488 goat anti-mouse IgG (H+L) used at a 1/1000 dilution for 1h. 10% Goat serum was used as the blocking agent for all blocking steps. DAPI was used to stain the cell nuclei (blue). Target protein locates mainly in mitochondria.
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WB Images
(click to enlarge)
Figure 2. 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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Flow Images
(click to enlarge)
Figure 3. HeLa cells were stained with 1 µg/mL TFP (HADHA) antibody (MS702) (blue) or an equal amount of an isotype control antibody (red) and analyzed by flow cytometry.
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