Anti-Iduronate 2 sulfatase antibody (ab113908)

Overview

  • Product name
    Anti-Iduronate 2 sulfatase antibody
    See all Iduronate 2 sulfatase primary antibodies
  • Description
    Rabbit polyclonal to Iduronate 2 sulfatase
  • Tested applications
    Suitable for: WBmore details
  • Species reactivity
    Reacts with: Human
    Predicted to work with: Mouse, Rat, Rabbit, Chicken, Guinea pig, Cow, Cat, Dog
  • Immunogen

    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).

  • Positive control
    • HeLa cell lysate

Properties

Applications

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.

Application Abreviews Notes
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.

Target

  • Function
    Required for the lysosomal degradation of heparan sulfate and dermatan sulfate.
  • Tissue specificity
    Liver, kidney, lung, and placenta.
  • Involvement in disease
    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.
  • Sequence similarities
    Belongs to the sulfatase family.
  • Post-translational
    modifications
    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.
  • Cellular localization
    Lysosome.
  • Information by UniProt
  • Database links
  • Alternative names
    • 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

Images

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

    Predicted band size : 62 kDa

References

ab113908 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"

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