Overview

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Accession
    • Species
      Human
    • Sequence
      MASPGCLWLLAVALLPWTCASRALQHLDPPAPLPLVIWHGMGDSCCNPLS MGAIKKMVEKKIPGIYVLSLEIGKTLMEDVENSFFLNVNSQVTTVCQALA KDPKLQQGYNAMGFSQGGQFLRAVAQRCPSPPMINLISVGGQHQGVFGLP RCPGESSHICDFIRKTLNAGAYSKVVQERLVQAEYWHDPIKEDVYRNHSI FLADINQERGINESYKKNLMALKKFVMVKFLNDSIVDPVDSEWFGFYRSG QAKETIPLQETSLYTQDRLGLKEMDNAGQLVFLATEGDHLQLSEEWFYAH IIPFLG
    • Molecular weight
      60 kDa including tags
    • Amino acids
      1 to 306

Specifications

Our Abpromise guarantee covers the use of ab116764 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • Applications

    ELISA

    SDS-PAGE

    Western blot

  • Form
    Liquid
  • Additional notes
    Protein concentration is above or equal to 0.05 mg/ml. Best use within three months from the date of receipt of this protein.
  • 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: 0.79% Tris HCl, 0.3% Glutathione

General Info

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

Images

  • 12.5% SDS-PAGE analysis of ab116764 at approximately 59.73kDa, stained with Coomassie Blue.

References

ab116764 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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