概述

  • 产品名称Anti-ACADVL抗体
    参阅全部 ACADVL 一抗
  • 描述
    兔多克隆抗体to ACADVL
  • 特异性Detects isoform 2.
  • 经测试应用适用于: WB, IHC-Pmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse, Rat, Rabbit, Horse, Guinea pig, Cow, Cat, Dog, Pig
  • 免疫原

    Synthetic peptide corresponding to a region within the N terminal amino acids 37-86 (RPYAGGAAQE SKSFAVGMFK GQLTTDQVFP YPSVLNEEQT QFLKELVEPV) of Human ACADVL, (NP_001029031).

  • 阳性对照
    • HepG2 cell lysate

应用

Our Abpromise guarantee covers the use of ab81497 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
WB Use a concentration of 1 µg/ml. Detects a band of approximately 64 kDa (predicted molecular weight: 64 kDa). Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.
IHC-P 1/100.

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 图像

  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) analysis of human pineal tissue labelling ACADVL with ab81497 at 1/100. A Cy3-conjugated donkey anti-rabbit IgG (1/200) was used as the secondary antibody. Positive staining shown in the cytoplasm of cell bodies of pinealocytes. Magnification: 20X. Exposure time: 0.5 - 2.0 seconds. Left - DAPI. Middle - ACADVL. Right - Merge.
  • Anti-ACADVL antibody (ab81497) at 1 µg/ml (in 5% skim milk / PBS buffer) + HepG2 cell lysate at 10 µg

    Secondary
    HRP conjugated anti-Rabbit IgG at 1/50000 dilution

    Predicted band size : 64 kDa
    Observed band size : 64 kDa
    Gel concentration 12%

Anti-ACADVL antibody (ab81497)参考文献

ab81497 has not yet been referenced specifically in any publications.

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