Overview

  • Product name

  • Description

    Mouse polyclonal to PPT1/PPT
  • Host species

    Mouse
  • Tested applications

    Suitable for: WB, IHC-Pmore details
  • Species reactivity

    Reacts with: Human
  • Immunogen

    Full length protein corresponding to Human PPT1/PPT.
    Database link: NP_000301.1

  • Positive control

    • WB: Human liver tissue lysate, PPT1/PPT transfected HEK-293T cell lysate. IHC-P: Human salivary gland tissue.
  • General notes

     This product was previously labelled as PPT1

     

Properties

Applications

Our Abpromise guarantee covers the use of ab89022 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
IHC-P
  • Application notes
    IHC-P: Use at a concentration of 3 µg/ml. Antigen retrieval is recommended Place sample in 1X citrate buffer (pH 6.0) and microwave at 750W for 20 minutes, cool sample
    WB: Use at a concentration of 1 µg/ml. Detects a band of approximately 34 kDa (predicted molecular weight: 34 kDa).


    Not yet tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • Target

    • Function

      Removes thioester-linked fatty acyl groups such as palmitate from modified cysteine residues in proteins or peptides during lysosomal degradation. Prefers acyl chain lengths of 14 to 18 carbons.
    • Involvement in disease

      Defects in PPT1 are the cause of neuronal ceroid lipofuscinosis type 1 (CLN1) [MIM:256730]. A form of neuronal ceroid lipofuscinosis with variable age at onset. Infantile, late-infantile, juvenile, and adult onset have been reported. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. The lipopigment pattern seen most often in CLN1 is referred to as granular osmiophilic deposits (GROD).
    • Sequence similarities

      Belongs to the palmitoyl-protein thioesterase family.
    • Cellular localization

      Lysosome.
    • Information by UniProt
    • Database links

    • Alternative names

      • Ceroid palmitoyl palmitoyl protein thioesterase 1 antibody
      • CLN1 antibody
      • EC 3.1.2.22 antibody
      • INCL antibody
      • Palmitoyl protein hydrolase 1 antibody
      • Palmitoyl protein thioesterase 1 antibody
      • Palmitoyl-protein hydrolase 1 antibody
      • Palmitoyl-protein thioesterase 1 antibody
      • PPT antibody
      • PPT-1 antibody
      • PPT1 antibody
      • PPT1_HUMAN antibody
      see all

    Images

    • Anti-PPT1/PPT antibody (ab89022) at 1 µg/ml + Human liver tissue lysate at 50 µg

      Predicted band size: 34 kDa
      Observed band size: 34 kDa

    • All lanes : Anti-PPT1/PPT antibody (ab89022) at 1 µg/ml

      Lane 1 : PPT1/PPT transfected HEK-293T cell lysate
      Lane 2 : Non-transfected lysate

      Lysates/proteins at 25 µg per lane.

      Predicted band size: 34 kDa
      Observed band size: 34 kDa

    • ab89022 at 3µg/ml staining PPT1/PPT in Human salivary gland by Immunohistochemistry, Formalin-fixed, Paraffin-embedded tissue.

    References

    This product has been referenced in:

    • Sima N  et al. Neural stem cells for disease modeling and evaluation of therapeutics for infantile (CLN1/PPT1) and late infantile (CLN2/TPP1) neuronal ceroid lipofuscinoses. Orphanet J Rare Dis 13:54 (2018). WB . Read more (PubMed: 29631617) »
    • Shyng C  et al. Widespread Expression of a Membrane-Tethered Version of the Soluble Lysosomal Enzyme Palmitoyl Protein Thioesterase-1. JIMD Rep 36:85-92 (2017). Read more (PubMed: 28213849) »
    See all 3 Publications for this product

    Customer reviews and Q&As

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