Recombinant Mouse Protective protein/Cathepsin A (PPCA) (His tag) (ab222429)

Overview

Description

  • Nature

    Recombinant
  • Source

    Baculovirus infected insect cells
  • Amino Acid Sequence
    • Accession
    • Species

      Mouse
    • Sequence

      APDQDEIDCLPGLAKQPSFRQYSGYLRASDSKHFHYWFVESQNDPKNSPV VLWLNGGPGCSSLDGLLTEHGPFLIQPDGVTLEYNPYAWNLIANVLYIES PAGVGFSYSDDKMYVTNDTEVAENNYEALKDFFRLFPEYKDNKLFLTGES YAGIYIPTLAVLVMQDPSMNLQGLAVGNGLASYEQNDNSLVYFAYYHGLL GNRLWTSLQTHCCAQNKCNFYDNKDPECVNNLLEVSRIVGKSGLNIYNLY APCAGGVPGRHRYEDTLVVQDFGNIFTRLPLKRRFPEALMRSGDKVRLDP PCTNTTAPSNYLNNPYVRKALHIPESLPRWDMCNFLVNLQYRRLYQSMNS QYLKLLSSQKYQILLYNGDVDMACNFMGDEWFVDSLNQKMEVQRRPWLVD YGESGEQVAGFVKECSHITFLTIKGAGHMVPTDKPRAAFTMFSRFLNKEP YVEHHHHHH
    • Molecular weight

      52 kDa including tags
    • Amino acids

      24 to 474
    • Tags

      His tag C-Terminus
    • Additional sequence information

      This product is the mature full length protein from aa 24 to 474. The signal peptide is not included (NP_001033581).

Specifications

Our Abpromise guarantee covers the use of ab222429 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

  • Endotoxin level

    < 1.000 Eu/µg
  • Purity

    > 90 % SDS-PAGE.
    ab222429 was purified using conventional chromatography techniques.
  • Form

    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    pH: 7.40
    Constituents: 10% Glycerol, PBS

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

  • 15% SDS-PAGE analysis of 3 µg ab222429.

    MWt 50-70 kDa (SDS-PAGE under reducing conditions).

References

ab222429 has not yet been referenced specifically in any publications.

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