Recombinant Human Protective protein/Cathepsin A (PPCA) (ab196068)

Description

  • Product name

    Recombinant Human Protective protein/Cathepsin A (PPCA)
    See all Protective protein/Cathepsin A (PPCA) proteins and peptides
  • Purity

    > 95 % SDS-PAGE.
    Purity greater than 95% as determined by SEC-HPLC and reducing SDS-PAGE.
  • Endotoxin level

    < 1.000 Eu/µg
  • Expression system

    HEK 293 cells
  • Accession

  • Protein length

    Full length protein
  • Animal free

    No
  • Nature

    Recombinant
    • Species

      Human
    • Sequence

      APDQDEIQRLPGLAKQPSFRQYSGYLKGSGSKHLHYWFVESQKDPENSPV VLWLNGGPGCSSLDGLLTEHGPFLVQPDGVTLEYNPYSWNLIANVLYLES PAGVGFSYSDDKFYATNDTEVAQSNFEALQDFFRLFPEYKNNKLFLTGES YAGIYIPTLAVLVMQDPSMNLQGLAVGNGLSSYEQNDNSLVYFAYYHGLL GNRLWSSLQTHCCSQNKCNFYDNKDLECVTNLQEVARIVGNSGLNIYNLY APCAGGVPSHFRYEKDTVVVQDLGNIFTRLPLKRMWHQALLRSGDKVRMD PPCTNTTAASTYLNNPYVRKALNIPEQLPQWDMCNFLVNLQYRRLYRSMN SQYLKLLSSQKYQILLYNGDVDMACNFMGDEWFVDSLNQKMEVQRRPWLV KYGDSGEQIAGFVKEFSHIAFLTIKGAGHMVPTDKPLAAFTMFSRFLNKQ PYHHHHHH
    • Predicted molecular weight

      52 kDa including tags
    • Amino acids

      29 to 480
    • Tags

      His tag C-Terminus

Specifications

Our Abpromise guarantee covers the use of ab196068 in the following tested applications.

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

  • Applications

    HPLC

    SDS-PAGE

  • Form

    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on Dry Ice. Store at -20°C. Avoid freeze / thaw cycle.

    pH: 7.40
    Constituents: 99% Phosphate Buffer, 0.87% Sodium chloride

General Info

  • Alternative names

    • beta galactosidase 2
    • BETA GALACTOSIDASE PROTECTIVE PROTEIN
    • beta-galactosidase 2
    • beta-galactosidase protective protein
    • betagalactosidase 2
    • Carboxypeptidase C
    • Carboxypeptidase L
    • carboxypeptidase Y-like kininase
    • Cathepsin A
    • Ctsa
    • deamidase
    • EC 3.4.16.5
    • Glactosialidosis
    • GLB2
    • Goldberg Syndrome
    • GSL
    • lysosomal carboxypeptidase A
    • Lysosomal protective protein
    • Lysosomal protective protein 20 kDa chain
    • Lysosomal protective protein deficiency
    • NEURAMINIDASE BETA GALACTOSIDASE EXPRESSION; NGBE
    • Neuraminidase deficiency with beta-galactosidase deficiency
    • NGBE
    • OTTHUMP00000031778
    • OTTHUMP00000031781
    • PPCA
    • PPCA deficiency
    • PPGB
    • PPGB_HUMAN
    • Protective protein cathepsin A
    • Protective protein for beta galactosidase
    • Protective protein for beta-galactosidase
    • Protective protein/cathepsin A deficiency
    • urinary kininase
    see all
  • Function

    Protective protein appears to be essential for both the activity of beta-galactosidase and neuraminidase, it associates with these enzymes and exerts a protective function necessary for their stability and activity. This protein is also a carboxypeptidase and can deamidate tachykinins.
  • Involvement in disease

    Defects in CTSA are the cause of galactosialidosis (GSL) [MIM:256540]. A lysosomal storage disease associated with a combined deficiency of beta-galactosidase and neuraminidase, secondary to a defect in cathepsin A. All patients have clinical manifestations typical of a lysosomal disorder, such as coarse facies, cherry red spots, vertebral changes, foam cells in the bone marrow, and vacuolated lymphocytes. Three phenotypic subtypes are recognized. The early infantile form is associated with fetal hydrops, edema, ascites, visceromegaly, skeletal dysplasia, and early death. The late infantile type is characterized by hepatosplenomegaly, growth retardation, cardiac involvement, and a normal or mildly affected mental state. The juvenile/adult form is characterized by myoclonus, ataxia, angiokeratoma, mental retardation, neurologic deterioration, absence of visceromegaly, and long survival.
  • Sequence similarities

    Belongs to the peptidase S10 family.
  • Cellular localization

    Lysosome.
  • Information by UniProt

References

ab196068 has not yet been referenced specifically in any publications.

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