概述

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

    Synthetic peptide corresponding to a region within C terminal amino acids 312-361 (YIFYTSDNGY HTGQFSLPID KRQLYEFDIK VPLLVRGPGI KPNQTSKMLV) of Rat GNS (NP_001011989).

  • 阳性对照
    • Rat heart lysate.

性能

应用

Our Abpromise guarantee covers the use of ab111441 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: 58 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.

靶标

  • 疾病相关Defects in GNS are the cause of mucopolysaccharidosis type 3D (MPS3D) [MIM:252940]; also known as Sanfilippo D syndrome. MPS3D is a form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years; severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life.
  • 序列相似性Belongs to the sulfatase family.
  • 翻译后修饰The form A (78 kDa) is processed by internal peptidase cleavage to a 32 kDa N-terminal species (form B) and a 48 kDa C-terminal species.
    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
    • 2610016K11Rik antibody
    • AU042285 antibody
    • C87209 antibody
    • G6S antibody
    • Glucosamine (N-acetyl) 6 sulfatase antibody
    • Glucosamine 6 sulfatase antibody
    • Glucosamine-6-sulfatase antibody
    • GNS antibody
    • GNS_HUMAN antibody
    • MGC21274 antibody
    • N acetylglucosamine 6 sulfatase [Precursor] antibody
    • N-acetylglucosamine-6-sulfatase antibody
    • N28088 antibody
    see all

Anti-GNS antibody 图像

  • Anti-GNS antibody (ab111441) at 1 µg/ml + Rat heart lysate at 10 µg

    Predicted band size : 58 kDa

Anti-GNS antibody (ab111441)参考文献

ab111441 has not yet been referenced specifically in any publications.

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