Overview

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Species
      Human
    • Sequence
      MGLQACLLGLFALILSGKCSYSPEPDQRRTLPPGWVSLGRADPEEELSLT FALRQQNVERLSELVQAVSDPSSPQYGKYLTLENVADLVRPSPLTLHTVQ KWLLAAGAQKCHSVITQDFLTCWLSIRQAELLLPGAEFHHYVGGPTETHV VRSPHPYQLPQALAPHVDFVGGLHRFPPTSSLRQRPEPQVTGTVGLHLGV TPSVIRKRYNLTSQDVGSGTSNNSQACAQFLEQYFHDSDLAQFMRLFGGN FAHQASVARVVGQQGRGRAGIEASLDVQYLMSAGANISTWVYSSPGRHEG QEPFLQWLMLLSNESALPHVHTVSYGDDEDSLSSAYIQRVNTELMKAAAR GLTLLFASGDSGAGCWSVSGRHQFRPTFPASSPYVTTVGGTSFQEPFLIT NEIVDYISGGGFSNVFPRPSYQEEAVTKFLSSSPHLPPSSYFNASGRAYP DVAALSDGYWVVSNRVPIPWVSGTSASTPVFGGILSLINEHRILSGRPPL GFLNPRLYQQHGAGLFDVTRGCHESCLDEEVEGQGFCSGPGWDPVTGWGT PNFPALLKTLLNP
    • Amino acids
      1 to 563
    • Tags
      proprietary tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab152286 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
    • Cell growth inhibiting gene 1 protein
    • Cell growth-inhibiting gene 1 protein
    • Ceroid lipofuscinosis neuronal 2
    • Ceroid lipofuscinosis neuronal 2 late infantile
    • Ceroid lipofuscinosis neuronal 2 late infantile (Jansky Bielschowsky disease)
    • CLN 2
    • CLN2
    • GIG 1
    • GIG1
    • Growth inhibiting protein 1
    • LPIC
    • Lysosomal pepstatin insensitive protease
    • Lysosomal pepstatin-insensitive protease
    • MGC21297
    • TPP 1
    • TPP I
    • TPP-1
    • TPP-I
    • Tpp1
    • TPP1_HUMAN
    • TPPI
    • Tripeptidyl aminopeptidase
    • Tripeptidyl peptidase I
    • Tripeptidyl-peptidase 1
    • Tripeptidyl-peptidase I
    see all
  • Function
    Lysosomal serine protease with tripeptidyl-peptidase I activity. May act as a non-specific lysosomal peptidase which generates tripeptides from the breakdown products produced by lysosomal proteinases. Requires substrates with an unsubstituted N-terminus.
  • Tissue specificity
    Detected in all tissues examined with highest levels in heart and placenta and relatively similar levels in other tissues.
  • Involvement in disease
    Defects in TPP1 are the cause of neuronal ceroid lipofuscinosis type 2 (CLN2) [MIM:204500]. A form of neuronal ceroid lipofuscinosis. 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 CLN2 consists of curvilinear profiles.
  • Sequence similarities
    Belongs to the peptidase S53 family.
  • Post-translational
    modifications
    Activated by autocatalytic proteolytical processing upon acidification. N-glycosylation is required for processing and activity.
  • Cellular localization
    Lysosome. Melanosome. Identified by mass spectrometry in melanosome fractions from stage I to stage IV.
  • Information by UniProt

Images

  • ab152286 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab152286 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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