概述

  • 产品名称Anti-ARH抗体
    参阅全部 ARH 一抗
  • 描述
    小鼠单克隆抗体to ARH
  • 经测试应用适用于: WB, ICC/IF, IHC-P, Flow Cytmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Recombinant full length protein, corresponding to amino acids 1-264 of Human ARH

性能

应用

Our Abpromise guarantee covers the use of ab57261 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 - 5 µg/ml.
ICC/IF Use a concentration of 10 µg/ml.
IHC-P Use a concentration of 2 µg/ml.
Flow Cyt Use 0.01µg for 106 cells. ab170190-Mouse monoclonal IgG1, is suitable for use as an isotype control with this antibody.

靶标

  • 功能Adapter protein (clathrin-associated sorting protein (CLASP)) required for efficient endocytosis of the LDL receptor (LDLR) in polarized cells such as hepatocytes and lymphocytes, but not in non-polarized cells (fibroblasts). May be required for LDL binding and internalization but not for receptor clustering in coated pits. May facilitate the endocytocis of LDLR and LDLR-LDL complexes from coated pits by stabilizing the interaction between the receptor and the structural components of the pits. May also be involved in the internalization of other LDLR family members. Binds to phosphoinositides, which regulate clathrin bud assembly at the cell surface.
  • 组织特异性Expressed at high levels in the kidney, liver, and placenta, with lower levels detectable in brain, heart, muscle, colon, spleen, intestine, lung, and leukocytes.
  • 疾病相关Defects in LDLRAP1 are the cause of autosomal recessive hypercholesterolemia (ARH) [MIM:603813]. ARH is a disorder caused by defective internalization of LDL receptors (LDLR) in the liver. ARH has the clinical features of familial hypercholesterolemia (FH) [MIM:143890] homozygotes, including severely elevated plasma LDL cholesterol, tuberous and tendon xanthomata, and premature atherosclerosis. LDL receptor (LDLR) activity measured in skin fibroblasts is normal, as the LDL binding ability.
  • 序列相似性Contains 1 PID domain.
  • 结构域The [DE]-X(1,2)-F-X-X-[FL]-X-X-X-R motif mediates interaction the AP-2 complex subunit AP2B1.
  • 翻译后修饰Phosphorylated upon DNA damage, probably by ATM or ATR.
  • 细胞定位Cytoplasm.
  • Information by UniProt
  • 数据库链接
  • 别名
    • ARH antibody
    • ARH GENE antibody
    • ARH_HUMAN antibody
    • ARH1 antibody
    • ARH2 antibody
    • Autosomal recessive hypercholesterolemia protein antibody
    • DKFZp586D0624 antibody
    • FHCB1 antibody
    • FHCB2 antibody
    • LDL receptor adaptor protein antibody
    • Ldlrap1 antibody
    • Low density lipoprotein receptor adapter protein 1 antibody
    • MGC34705 antibody
    • OTTHUMP00000008526 antibody
    see all

Anti-ARH antibody 图像

  • Overlay histogram showing HEK293 cells stained with ab57261 (red line). The cells were fixed with 80% methanol (5 min) and then permeabilized with 0.1% PBS-Tween for 20 min. The cells were then incubated in 1x PBS / 10% normal goat serum / 0.3M glycine to block non-specific protein-protein interactions followed by the antibody (ab57261, 0.01μg/1x106 cells) for 30 min at 22°C. The secondary antibody used was Alexa Fluor® 488 goat anti-mouse IgG (H&L) (ab150113) at 1/2000 dilution for 30 min at 22°C. Isotype control antibody (black line) was mouse IgG1 [ICIGG1] (ab91353, 1μg/1x106 cells) used under the same conditions. Unlabelled sample (blue line) was also used as a control. Acquisition of >5,000 events were collected using a 20mW Argon ion laser (488nm) and 525/30 bandpass filter. This antibody gave a positive signal in HEK293 cells fixed with 4% paraformaldehyde (10 min) permeabilized with 0.1% PBS-Tween for 20 min used under the same conditions.
  • ARH antibody (ab57261) used in immunofluorescence at 10ug/ml on HeLa cells.
  • ARH antibody (ab57261) used in immunohistochemistry at 2ug/ml on formalin fixed and paraffin embedded human maligant fibrous histiocytoma tissue.
  • ARH antibody (ab57261) at 1ug/lane + HeLa cell lysate at 25ug/lane.

Anti-ARH antibody (ab57261)参考文献

This product has been referenced in:
  • Poirier S  et al. Dissection of the endogenous cellular pathways of PCSK9-induced low density lipoprotein receptor degradation: evidence for an intracellular route. J Biol Chem 284:28856-64 (2009). Read more (PubMed: 19635789) »

See 1 Publication for this product

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