Overview

  • Product name
  • Description
    Rabbit polyclonal to ARSA
  • Host species
    Rabbit
  • Tested applications
    Suitable for: WBmore details
  • Species reactivity
    Reacts with: Human
    Predicted to work with: Mouse, Rat, Cow, Dog
  • Immunogen

    Recombinant fragment, corresponding to a region within internal sequence amino acids 169-405 of Human ARSA

  • Positive control
    • A431; H1299; HeLa and HepG2 cell lysates.

Properties

Applications

Our Abpromise guarantee covers the use of ab154300 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 1/500 - 1/3000. 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 antibody (ab154300) at 1/1000 dilution

    Lane 1 : H1299 whole cell lysate
    Lane 2 : HeLa whole cell lysate

    Lysates/proteins at 30 µg per lane.

    Predicted band size: 54 kDa



    10% SDS PAGE

References

ab154300 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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