Anti-Protective protein/Cathepsin A (PPCA) antibody (ab126127)

Overview

  • Product name
    Anti-Protective protein/Cathepsin A (PPCA) antibody
    See all Protective protein/Cathepsin A (PPCA) primary antibodies
  • Description
    Rabbit polyclonal to Protective protein/Cathepsin A (PPCA)
  • Tested applications
    Suitable for: WB, IHC-Pmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

    Recombinant fragment, corresponding to a region within amino acids 124-426 of Human Protective protein/Cathepsin A (PPCA) (P10619).

  • Positive control
    • HeLa and HepG2 whole cell lysates; 59T xenograft tissue.
  • General notes
    Keep as a concentrated solution.

Properties

Applications

Our Abpromise guarantee covers the use of ab126127 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 1/1000 - 1/10000. Predicted molecular weight: 54 kDa.
IHC-P 1/100 - 1/500. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

Target

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

Images

  • ab126127, at 1/500 dilution, staining Protective protein/Cathepsin A (PPCA) in paraffin embedded 59T xenograft tissue by Immunohistochemistry.
  • All lanes : Anti-Protective protein/Cathepsin A (PPCA) antibody (ab126127) at 1/5000 dilution

    Lane 1 : HeLa whole cell lysate
    Lane 2 : HepG2 whole cell lysate

    Lysates/proteins at 30 µg per lane.


    Predicted band size : 54 kDa

References

ab126127 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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