Anti-Iduronate 2 sulfatase抗体(ab113908)

概述

  • 产品名称Anti-Iduronate 2 sulfatase抗体
    参阅全部 Iduronate 2 sulfatase 一抗
  • 描述
    兔多克隆抗体to Iduronate 2 sulfatase
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse, Rat, Rabbit, Chicken, Guinea pig, Cow, Cat, Dog
  • 免疫原

    Synthetic peptide, corresponding to a region within N terminal amino acids 154-203 (SFPPYHPSSE KYENTKTCRG DGELHANLLC VDVLDVPEGT PDKQSTE) of Human Iduronate 2 sulfatase (NP_000193).

  • 阳性对照
    • HeLa cell lysate

性能

相关产品

应用

Our Abpromise guarantee covers the use of ab113908 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. Predicted molecular weight: 62 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.

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

Anti-Iduronate 2 sulfatase antibody 图像

  • Anti-Iduronate 2 sulfatase antibody (ab113908) at 1 µg/ml + HeLa cell lysate at 10 µg

    Predicted band size : 62 kDa

Anti-Iduronate 2 sulfatase antibody (ab113908)参考文献

ab113908 has not yet been referenced specifically in any publications.

Product Wall

There are currently no Abreviews or Questions for ab113908.
Please use the links above to contact us or submit feedback about this product.

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