(MS707)
Anti-ACADVL antibody [6A9AF2] (ab110285)
Overview
- Product nameAnti-ACADVL antibody [6A9AF2]See all ACADVL primary antibodies ...
- DescriptionMouse monoclonal [6A9AF2] to ACADVL
- Tested applicationsICC, IP, IHC-P more details
- Species reactivityReacts with: Mouse, Rat, Cow, Human
- Immunogen
Human liver mitocondria
- Positive controlHuman HDFn cells; Human liver mitochondria; Human cerebellum tissue.
Properties
- FormLiquid
- Storage instructionsStore at +4°C. Do not freeze.
- Storage bufferPreservative: 0.02% Sodium azide
Constituent: HBS -
Concentration information loading... - Purity>95% by SDS-PAGE
- Purification notesThe antibody was produced in vitro using hybridoma grown in serum-free media and then purified by biochemical fractionation.
- Clonality Monoclonal
- Clone number6A9AF2
- IsotypeIgG1
- Light chain typekappa
- Research Areas
Applications
Our Abpromise guarantee covers the use of ab110285 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
| Application | Notes |
|---|---|
| ICC | ICC: Use a concentration of 5 µg/ml. |
| IP | IP: Use at an assay dependent dilution. |
| IHC-P | IHC-P: 1/250. Perform heat mediated antigen retrieval - 1 min pressure cook in 1mmol EDTA pH8. |
Target
- FunctionActive toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accomodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons.
- PathwayLipid metabolism; mitochondrial fatty acid beta-oxidation.
- Involvement in diseaseDefects in ACADVL are the cause of acyl-CoA dehydrogenase very long chain deficiency (ACADVLD) [MIM:201475]. ACADVLD is an autosomal recessive disease which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form, with early onset, high mortality, and high incidence of cardiomyopathy; a milder childhood form, with later onset, usually with hypoketotic hypoglycemia as the main presenting feature, low mortality, and rare cardiomyopathy; and an adult form, with isolated skeletal muscle involvement, rhabdomyolysis, and myoglobinuria, usually triggered by exercise or fasting.
- Sequence similaritiesBelongs to the acyl-CoA dehydrogenase family.
- Cellular localizationMitochondrion inner membrane.
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Database links
- Entrez Gene: 282130 Cow
- Entrez Gene: 37 Human
- Entrez Gene: 11370 Mouse
- Entrez Gene: 25363 Rat
- Omim: 609575 Human
- SwissProt: P49748 Human
- SwissProt: P50544 Mouse
- SwissProt: P45953 Rat
- Unigene: 437178 Human
- Unigene: 18630 Mouse
- Unigene: 33319 Rat
see all
Target information above from: UniProt accession
P49748
The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010)
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Alternative names
- ACAD 6 antibodyACAD6 antibodyACADV_HUMAN antibody
- Acadvl antibodyAcyl CoA dehydrogenase very long chain antibodyAcyl Coenzyme A dehydrogenase very long chain antibodyLCACD antibodymitochondrial antibodyVery long chain specific acyl CoA dehydrogenase antibodyVery long chain specific acyl CoA dehydrogenase mitochondrial antibodyVery long-chain specific acyl-CoA dehydrogenase antibodyVLCAD antibody
see all
Anti-ACADVL antibody [6A9AF2] images
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Immunocytochemistry image of ACADVL antibody [6A9AF2] (ab110285) 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 ab110285 at 5 µg/ml for 2h at room temperature or over night at 4°C. The secondary antibody was (red) Alexa Fluor® 594 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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Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-ACADVL antibody [6A9AF2] (ab110285)ab110285,at 1/250 dilution, staining ACADVL in formalin-fixed, paraffin-embedded human cerebellum tissue. Immunoactivity is most intense in neuronal cell bodies, most notably in the large Purkinje cells. -
ACADVL immunocaptured from 0.75mg of human liver mitochondria lysate /10ul antibody conjugated beads using ab110285.
References for Anti-ACADVL antibody [6A9AF2] (ab110285)
This product has been referenced in:
- Sweeney TE et al. A toll-like receptor 2 pathway regulates the Ppargc1a/b metabolic co-activators in mice with Staphylococcal aureus sepsis. PLoS One 6:e25249 (2011). WB ; Mouse . Read more (PubMed: 21966468) »
![Immunocytochemistry/ Immunofluorescence - Anti-ACADVL antibody [6A9AF2] (ab110285) Immunocytochemistry/ Immunofluorescence - Anti-ACADVL antibody [6A9AF2] (ab110285)](http://a.abcam.com/ps/datasheet/images/110/ab110285/ACADVL-Primary-antibodies-ab110285-6.gif)
![Immunoprecipitation - Anti-ACADVL antibody [6A9AF2] (ab110285) Immunoprecipitation - Anti-ACADVL antibody [6A9AF2] (ab110285)](http://a.abcam.com/ps/datasheet/images/110/ab110285/ACADVL-Primary-antibodies-ab110285-9.gif)