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Products:Tags & Cell Markers >> Subcellular Markers >> Organelles >> Mitochondria
Overview
Product name
Anti-HADHA antibody
See all HADHA products (3) ...
Description
Rabbit polyclonal to HADHA
Tested applications
IHC-P, ICC/IF, WBmore details
Cross reactivity
Reacts with
Mouse, Rat, Human
Immunogen
Synthetic peptide surrounding amino acid 750 (Human)
Positive control
Jurkat cell lysate 3T3 cell lysate Rat kidney lysate
Properties
Form
Liquid
Storage instructions
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Storage buffer
Preservative: 0.01% Thimerosal (merthiolate)
Constituents: 30% Glycerol, 0.5% BSA, PBS
Concentration
Concentration information loading...
Purity
Protein A purified
Clonality
Polyclonal
Isotype
IgG
Research areas
Cancer >> Cancer Metabolism >> Metabolic signaling pathway >> Metabolism of lipids and lipoproteins
Cardiovascular >> Lipids / Lipoproteins >> Fatty Acids >> Metabolism
Signal Transduction >> Metabolism >> Lipid metabolism
Signal Transduction >> Metabolism >> Mitochondrial
Tags & Cell Markers >> Subcellular Markers >> Organelles >> Mitochondria
Applications
Show applications key
Our Abpromise guarantee covers the use of ab54477 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
IHC-P: Use a concentration of 1 µg/mlPerform heat mediated antigen retrieval before commencing with IHC staining protocol.
ICC/IF: Use a concentration of 1 µg/ml
WB: Use a concentration of 0.5 - 4 µg/ml.Detects a band of approximately 83 kDa (predicted molecular weight: 83 kDa).
Target
Function
Bifunctional subunit.
Pathway
Lipid metabolism; fatty acid beta-oxidation.
Involvement in disease
Defects in HADHA are a cause of trifunctional protein deficiency (TFP deficiency) [MIM:609015]. The clinical manifestations are very variable and include hypoglycemia, cardiomyopathy and sudden death. Phenotypes with mainly hepatic and neuromyopathic involvement can also be distinguished. Biochemically, TFP deficiency is defined by the loss of all enzyme activities of the TFP complex.
Defects in HADHA are the cause of long-chain 3-hydroxyl-CoA dehydrogenase deficiency (LCHAD deficiency) [MIM:609016]. The clinical features are very similar to TFP deficiency. Biochemically, LCHAD deficiency is characterized by reduced long-chain 3-hydroxyl-CoA dehydrogenase activity, while the other enzyme activities of the TFP complex are normal or only slightly reduced.
Defects in HADHA are a cause of maternal acute fatty liver of pregnancy (AFLP) [MIM:609016]. AFLP is a severe maternal illness occurring during pregnancies with affected fetuses. This disease is associated with LCHAD deficiency and characterized by sudden unexplained infant death or hypoglycemia and abnormal liver enzymes (Reye-like syndrome).
Sequence similarities
In the N-terminal section; belongs to the enoyl-CoA hydratase/isomerase family.
In the central section; belongs to the 3-hydroxyacyl-CoA dehydrogenase family.
Cellular localization
Mitochondrion.
Target information above from: UniProt accessionP40939
The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010).
Alternative names
- 3 ketoacyl Coenzyme A (CoA) thiolase alpha subunit antibody
- 3 oxoacyl CoA thiolase antibody
- 78 kDa gastrin binding protein antibody
see all
Database links
- Entrez Gene: 3030 Human
- Entrez Gene: 97212 Mouse
- Entrez Gene: 170670 Rat
- SwissProt: P40939 Human
- SwissProt: Q8BMS1 Mouse
- SwissProt: Q64428 Rat
- Unigene: 516032 Human
- Unigene: 200497 Mouse
see all
Anti-HADHA antibody images:
Western blot - TFPI antibody (ab54477)
All lanes : Anti-HADHA antibody (ab54477) at 4 µg/ml
Lane 1 : Jurkat cell lysate 30-50 ug/lane.
Lane 2 : Jurkat cell lysate 30-50 ug/lane.
Lane 3 : 3T3 cell lysate 30-50 ug/lane.
Lane 4 : Rat kidney lysate 30-50 ug/lane.
Secondary
Anti-Rabbit IgG, HRP-Linked Antibody at 1/5000 dilution
Predicted band size : 83 kDa
Observed band size : 83 kDa
Immunocytochemistry/ Immunofluorescence-HADHA antibody(ab54477)
ICC/IF image of ab54477 stained HepG2 cells. The cells were 100% methanol fixed (5 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody (ab54477, 1µg/ml) overnight at +4°C. The secondary antibody (green) was Alexa Fluor® 488 goat anti-rabbit IgG (H+L) used at a 1/1000 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)-HADHA antibody(ab54477)
ab54477 (1µg/ml) staining HADHA in human ileum using an automated system (DAKO Autostainer Plus). Using this protocol there is strong mitochondrial staining.
Sections were rehydrated and antigen retrieved with the Dako 3 in 1 AR buffer EDTA pH 9.0 in a DAKO PT Link. Slides were peroxidase blocked in 3% H2O2 in methanol for 10 mins. They were then blocked with Dako Protein block for 10 minutes (containing casein 0.25% in PBS) then incubated with primary antibody for 20 min and detected with Dako Envision Flex amplification kit for 30 minutes. Colorimetric detection was completed with Diaminobenzidine for 5 minutes. Slides were counterstained with Haematoxylin and coverslipped under DePeX. Please note that, for manual staining, optimization of primary antibody concentration and incubation time is recommended. Signal amplification may be required.
References for Anti-HADHA antibody (ab54477)
This product has been referenced in:
- Schulz Net al. Role of Medium- and Short-Chain L-3-Hydroxyacyl-CoA Dehydrogenase in the Regulation of Body Weight and Thermogenesis. Endocrinology 152:4641-51 (2011).Read more (PubMed: 21990309) »
- Ebmeier CC & Taatjes DJ Activator-Mediator binding regulates Mediator-cofactor interactions. Proc Natl Acad Sci U S A 107:11283-8 (2010). WB; Human.Read more (PubMed: 20534441) »
See all 2 publications for this product
Publishing research using ab54477? Please let us know so that we can cite the reference in this datasheet
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"