概述

  • 产品名称Anti-HSPC014抗体
    参阅全部 HSPC014 一抗
  • 描述
    鸡多克隆抗体to HSPC014
  • 经测试应用适用于: IHC-Fr, ELISA, WBmore details
  • 种属反应性
    与反应: Mouse, Rat, Human
  • 免疫原

    Synthetic peptide, corresponding to amino acids 1-141 of Human HSPC014.

性能

应用

Our Abpromise guarantee covers the use of ab16424 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
IHC-Fr Use at an assay dependent dilution. PubMed: 20226437
ELISA Use at an assay dependent dilution.
WB 1/1000 - 1/2000. Predicted molecular weight: 17 kDa.

靶标

  • 功能Molecular chaperone essential for the assembly of standard proteasomes and immunoproteasomes. Degraded after completion of proteasome maturation. Mediates the association of 20S preproteasome with the endoplasmic reticulum.
  • 组织特异性Strongly expressed from the basal layer to the granular layer of healthy epidermis, whereas in KLICK patients there is a gradual decrease of expression toward the granular layer.
  • 疾病相关Defects in POMP are the cause of keratosis linearis with ichthyosis congenita and sclerosing keratoderma (KLICK) [MIM:601952]. KLICK is a keratinizing disorder characterized by ichthyosis, palmoplantar keratoderma with constricting bands around fingers, flexural deformities of fingers and keratotic papules in a linear distribution on the flexural side of large joints. Histological examination of the skin of affected individuals shows hypertrophy and hyperplasia of the spinous, granular and horny epidermal layer.
  • 序列相似性Belongs to the POMP/UMP1 family.
  • 细胞定位Cytoplasm > cytosol. Nucleus. Microsome membrane.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 2510048O06Rik antibody
    • C13orf12 antibody
    • Chromosome 13 open reading frame 12 antibody
    • HSPC 014 antibody
    • HSPC036 protein antibody
    • hUMP 1 antibody
    • hUMP1 antibody
    • PNAS 110 antibody
    • PNAS110 antibody
    • Pomp antibody
    • POMP_HUMAN antibody
    • Proteasome maturation protein antibody
    • Proteassemblin antibody
    • Protein UMP1 homolog antibody
    • UMP 1 antibody
    • UMP1 antibody
    • UMP1, yeast, homolog of antibody
    • Voltage gated K channel beta subunit 4.1 antibody
    • Voltage-gated K channel beta subunit 4.1 antibody
    • voltage-gated potassium channel beta subunit 4.1 antibody
    see all

Anti-HSPC014 antibody (ab16424)参考文献

This product has been referenced in:
  • Dahlqvist J  et al. A single-nucleotide deletion in the POMP 5' UTR causes a transcriptional switch and altered epidermal proteasome distribution in KLICK genodermatosis. Am J Hum Genet 86:596-603 (2010). IHC-Fr ; Human . Read more (PubMed: 20226437) »

See 1 Publication for this product

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