Recombinant Human Protective protein/Cathepsin A (PPCA) (ab159166)

Overview

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Species
      Human
    • Sequence
      MIRAAPPPLFLLLLLLLLLVSWASRGEAAPDQDEIQRLPGLAKQPSFRQY SGYLKGSGSKHLHYWFVESQKDPENSPVVLWLNGGPGCSSLDGLLTEHGP FLVQPDGVTLEYNPYSWNLIANVLYLESPAGVGFSYSDDKFYATNDTEVA QSNFEALQDFFRLFPEYKNNKLFLTGESYAGIYIPTLAVLVMQDPSMNLQ GLAVGNGLSSYEQNDNSLVYFAYYHGLLGNRLWSSLQTHCCSQNKCNFYD NKDLECVTNLQEVARIVGNSGLNIYNLYAPCAGGVPSHFRYEKDTVVVQD LGNIFTRLPLKRMWHQALLRSGDKVRMDPPCTNTTAASTYLNNPYVRKAL NIPEQLPQWDMCNFLVNLQYRRLYRSMNSQYLKLLSSQKYQILLYNGDVD MACNFMGDEWFVDSLNQKMEVQRRPWLVKYGDSGEQIAGFVKEFSHIAFL TIKGAGHMVPTDKPLAAFTMFSRFLNKQPY
    • Amino acids
      1 to 480
    • Tags
      proprietary tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab159166 in the following tested applications.

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

  • Applications

    ELISA

    Western blot

  • Form
    Liquid
  • Additional notes
    Protein concentration is above or equal to 0.05 mg/ml.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl

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

Images

  • ab159166 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab159166 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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