Anti-Iduronate 2 sulfatase抗体(ab70025)

概述

  • 产品名称
    Anti-Iduronate 2 sulfatase抗体
    参阅全部 Iduronate 2 sulfatase 一抗
  • 描述
    小鼠多克隆抗体to Iduronate 2 sulfatase
  • 经测试应用
    适用于: WBmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Full length human Iduronate 2 sulfatase (NP_000193.1, aa 1 - 550)

  • 阳性对照
    • Iduronate 2 sulfatase transfected 293T lysate.

性能

应用

Our Abpromise guarantee covers the use of ab70025 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 1/500 - 1/1000. Detects a band of approximately 75 kDa (predicted molecular weight: 62 kDa).

靶标

  • 功能
    Required for the lysosomal degradation of heparan sulfate and dermatan sulfate.
  • 组织特异性
    Liver, kidney, lung, and placenta.
  • 疾病相关
    Defects in IDS are the cause of mucopolysaccharidosis type 2 (MPS2) [MIM:309900]; also known as Hunter syndrome. MPS2 is an X-linked lysosomal storage disease characterized by intracellular accumulation of heparan sulfate and dermatan sulfate and their excretion in urine. Most children with MPS2 have a severe form with early somatic abnormalities including skeletal deformities, hepatosplenomegaly, and progressive cardiopulmonary deterioration. A prominent feature is neurological damage that presents as developmental delay and hyperactivity but progresses to mental retardation and dementia. They die before 15 years of age, usually as a result of obstructive airway disease or cardiac failure. In contrast, those with a mild form of MPS2 may survive into adulthood, with attenuated somatic complications and often without mental retardation.
  • 序列相似性
    Belongs to the sulfatase family.
  • 翻译后修饰
    The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity.
  • 细胞定位
    Lysosome.
  • Information by UniProt
  • 数据库链接
  • 别名
    • Alpha L iduronate sulfate sulfatase antibody
    • Alpha-L-iduronate sulfate sulfatase antibody
    • AW214631 antibody
    • Ids antibody
    • IDS_HUMAN antibody
    • Iduronate 2 sulfatase 14 kDa chain antibody
    • Iduronate 2 sulfatase 42 kDa chain antibody
    • Iduronate 2 sulfatase antibody
    • Iduronate 2-sulfatase 14 kDa chain antibody
    • Iduronate sulfatase antibody
    • Idursulfase antibody
    • MPS2 antibody
    • RP23-29M4.1 antibody
    • SIDS antibody
    see all

图片

  • All lanes : Anti-Iduronate 2 sulfatase antibody (ab70025) at 1/500 dilution

    Lane 1 : Iduronate 2 sulfatase transfected 293T lysate
    Lane 2 : Non transfected 293T lysate

    Lysates/proteins at 25 µg per lane.

    Secondary
    Goat Anti-Mouse IgG (H&L)-HRP at 1/2500 dilution

    Predicted band size : 62 kDa
    Observed band size : 75 kDa (why is the actual band size different from the predicted?)

实验方案

文献

ab70025 has not yet been referenced specifically in any publications.

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