Overview

  • Product name

  • Description

    Rabbit polyclonal to ARSA/ASA
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WBmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse, Rat
  • Immunogen

    Synthetic peptide corresponding to Human ARSA/ASA aa 400-500 conjugated to keyhole limpet haemocyanin.
    (Peptide available as ab88153)

  • Positive control

    • This antibody gave a positive signal in the following lysates: Human Testis Tissue Lysate Jurkat whole cell lysate HepG2 Whole Cell Lysate
  • General notes

    Previously labelled as ARSA. 

Properties

Applications

Our Abpromise guarantee covers the use of ab76890 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. Detects a band of approximately 54 kDa (predicted molecular weight: 54 kDa).

Target

  • Function

    Hydrolyzes cerebroside sulfate.
  • Involvement in disease

    Defects in ARSA are a cause of leukodystrophy metachromatic (MLD) [MIM:250100]. MLD is a disease due to a lysosomal storage defect. It is characterized by intralysosomal storage of cerebroside-3-sulfate in neural and non-neural tissues, with a diffuse loss of myelin in the central nervous system. Progressive demyelination causes a variety of neurological symptoms, including gait disturbances, ataxias, optical atrophy, dementia, seizures, and spastic tetraparesis. Three forms of the disease can be distinguished according to the age at onset: late-infantile, juvenile and adult.
    Arylsulfatase A activity is defective in multiple sulfatase deficiency (MSD) [MIM:272200]. MSD is a disorder characterized by decreased activity of all known sulfatases. MSD is due to defects in SUMF1 resulting in the lack of post-translational modification of a highly conserved cysteine into 3-oxoalanine. It combines features of individual sulfatase deficiencies such as metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay.
  • 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. This post-translational modification is severely defective in multiple sulfatase deficiency (MSD).
  • Cellular localization

    Lysosome.
  • Information by UniProt
  • Database links

  • Alternative names

    • arsA antibody
    • ARSA_HUMAN antibody
    • arylsulfatase A antibody
    • Arylsulfatase A component C antibody
    • As 2 antibody
    • AS A antibody
    • As2 antibody
    • ASA antibody
    • AW212749 antibody
    • C230037L18Rik antibody
    • Cerebroside-sulfatase antibody
    • metachromatic leucodystrophy antibody
    • MGC125207 antibody
    • MLD antibody
    • OTTHUMP00000196546 antibody
    • OTTHUMP00000196548 antibody
    • TISP73 antibody
    see all

Images

  • All lanes : Anti-ARSA/ASA antibody (ab76890) at 1 µg/ml

    Lane 1 : Human testis tissue lysate - total protein (ab30257)
    Lane 2 : Jurkat (Human T cell lymphoblast-like cell line) Whole Cell Lysate
    Lane 3 : HepG2 (Human hepatocellular liver carcinoma cell line) Whole Cell Lysate

    Lysates/proteins at 10 µg per lane.

    Secondary
    All lanes : Goat polyclonal to Rabbit IgG - H&L - Pre-Adsorbed (HRP) at 1/3000 dilution

    Developed using the ECL technique.

    Performed under reducing conditions.

    Predicted band size: 54 kDa
    Observed band size: 53 kDa
    why is the actual band size different from the predicted?


    Exposure time: 30 seconds

References

ab76890 has not yet been referenced specifically in any publications.

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