Overview

  • Product name

    Recombinant Human ARSA / ASA protein
  • Protein length

    Protein fragment

Description

  • Nature

    Recombinant
  • Source

    HEK 293 cells
  • Amino Acid Sequence
    • Accession
    • Species

      Human
    • Sequence

      RPPNIVLIFA DDLGYGDLGC YGHPSSTTPN LDQLAAGGLR FTDFYVPVSL CTPSRAALLT GRLPVRMGMY PGVLVPSSRG GLPLEEVTVA EVLAARGYLT GMAGKWHLGV GPEGAFLPPH QGFHRFLGIP YSHDQGPCQN LTCFPPATPC DGGCDQGLVP IPLLANLSVE AQPPWLPGLE ARYMAFAHDL MADAQRQDRP FFLYYASHHT HYPQFSGQSF AERSGRGPFG DSLMELDAAV GTLMTAIGDL GLLEETLVIF TADNGPETMR MSRGGCSGLL RCGKGTTYEG GVREPALAFW PGHIAPGVTH ELASSLDLLP TLAALAGAPL PNVTLDGFDL SPLLLGTGKS PRQSLFFYPS YPDEVRGVFA VRSGKYKAHF FTQGSAHSDT TADPACHASS SLTAHEPPLL YDLSKDPGEN YNLLGGVAGA TPEVLQALKQ LQLLKAQLDA AVTFGPSQVA RGEDPALQIC CHPGCTPRPA CCHCPDPHAV DHHHHHH
    • Molecular weight

      53 kDa including tags
    • Amino acids

      19 to 507
    • Tags

      His tag C-Terminus

Specifications

Our Abpromise guarantee covers the use of ab151369 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • Applications

    SDS-PAGE

    HPLC

  • Endotoxin level

    < 1.000 Eu/µg
  • Purity

    > 95 % SDS-PAGE.

  • Form

    Liquid
  • Additional notes

    Previously labelled as ARSA. 

  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Upon delivery aliquot. Store at -80°C. Avoid freeze / thaw cycle.

    pH: 7.50
    Constituents: 0.32% Tris HCl, 0.88% Sodium chloride

General Info

  • Alternative names

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

References

ab151369 has not yet been referenced specifically in any publications.

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