MitoSciences (MS707)

Anti-ACADVL antibody [6A9AF2] (ab110285)

Overview

  • Product nameAnti-ACADVL antibody [6A9AF2]See all ACADVL primary antibodies ...
  • Description
    Mouse monoclonal [6A9AF2] to ACADVL
  • Tested applicationsICC, IP, IHC-Pmore details
  • Species reactivity
    Reacts with: Mouse, Rat, Cow, Human
  • Immunogen

    Human liver mitocondria

  • Positive control
    • Human HDFn cells; Human liver mitochondria; Human cerebellum tissue.

Properties

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 Abreviews Notes
ICC Use a concentration of 5 µg/ml.
IP Use at an assay dependent dilution.
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.
  • Information by UniProt
  • Database links
  • Alternative names
    • ACAD 6 antibody
    • ACAD6 antibody
    • ACADV_HUMAN antibody
    • Acadvl antibody
    • Acyl CoA dehydrogenase very long chain antibody
    • Acyl Coenzyme A dehydrogenase very long chain antibody
    • LCACD antibody
    • mitochondrial antibody
    • Very long chain specific acyl CoA dehydrogenase antibody
    • Very long chain specific acyl CoA dehydrogenase mitochondrial antibody
    • Very long-chain specific acyl-CoA dehydrogenase antibody
    • VLCAD antibody
    see all

Anti-ACADVL antibody [6A9AF2] images

  • 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.
  • 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) »

See 1 Publication for this product

Product Wall

Thank you for your reply.

For UNTESTED applications, we have established a testing discount program. Here is a brief description of how it works:

The testing discount program is for customers who like to use an antibody/protein on...

Read More

Thank you for contacting us. I wish to apologize for the wait.

We really do not know what you would expect to see with this product in EM, e.g. linear arrays probes along the inner membrane. Regarding whether this product should work with g...

Read More

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"