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)
  • Host species

    Rabbit
  • Tested applications

    Suitable for: IHC-P, WBmore details
  • Species reactivity

    Reacts with: Mouse, Rat, Human
  • Immunogen

    Synthetic peptide within Human Protective protein/Cathepsin A (PPCA) aa 430-480 conjugated to keyhole limpet haemocyanin. The exact sequence is proprietary.
    Sequence:

    YGDSGEQIAGF VKEFSHIAFL TIKGAGHMVP TDKPLAAFTM FSRFLNKQPY


    Database link: P10619

  • Positive control

    • Rat liver tissue; Mouse testis and 293T lysates.

Properties

Applications

Our Abpromise guarantee covers the use of ab217857 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
IHC-P 1/100 - 1/500.
WB 1/100 - 1/1000. Predicted molecular weight: 54 kDa.

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

  • Immunohistochemical analysis of formalin-fixed, paraffin-embedded rat liver tissue labeling Protective protein/Cathepsin A (PPCA) with ab217857 at 1/200 dilution followed by conjugation to the secondary antibody and DAB staining.

  • Anti-Protective protein/Cathepsin A (PPCA) antibody (ab217857) at 1/300 dilution + Mouse testis lysates

    Predicted band size: 54 kDa

  • Anti-Protective protein/Cathepsin A (PPCA) antibody (ab217857) at 1/300 dilution + 293T lysates

    Predicted band size: 54 kDa

References

ab217857 has not yet been referenced specifically in any publications.

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