概述

  • 产品名称Anti-ACADVL抗体[6A9AF2]
    参阅全部 ACADVL 一抗
  • 描述
    小鼠单克隆抗体[6A9AF2] to ACADVL
  • 经测试应用适用于: ICC, IP, IHC-Pmore details
  • 种属反应性
    与反应: Mouse, Rat, Cow, Human
  • 免疫原

    Human liver mitocondria

  • 阳性对照
    • Human HDFn cells; Human liver mitochondria; Human cerebellum tissue.
  • 常规说明

    Product was previously marketed under the MitoSciences sub-brand.

性能

应用

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.

应用 Ab评论 说明
ICC Use a concentration of 5 µg/ml.
IP Use at an assay dependent concentration.
IHC-P 1/250. Perform heat mediated antigen retrieval - 1 min pressure cook in 1mmol EDTA pH8.

靶标

  • 功能Active 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.
  • 通路Lipid metabolism; mitochondrial fatty acid beta-oxidation.
  • 疾病相关Defects 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.
  • 序列相似性Belongs to the acyl-CoA dehydrogenase family.
  • 细胞定位Mitochondrion inner membrane.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 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] 图像

  • 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.

Anti-ACADVL antibody [6A9AF2] (ab110285)参考文献

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

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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...

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