Overview

Description

  • NatureRecombinant
  • SourceWheat germ
  • Amino Acid Sequence
    • AccessionP50897
    • SpeciesHuman
    • SequenceMASPGCLWLLAVALLPWTCASRALQHLDPPAPLPLVIWHGMGDSCCNPLS MGAIKKMVEKKIPGIYVLSLEIGKTLMEDVENSFFLNVNSQVTTVCQALA KDPKLQQGYNAMGFSQGGQFLRAVAQRCPSPPMINLISVGGQHQGVFGLP RCPGESSHICDFIRKTLNAGAYSKVVQERLVQAEYWHDPIKEDVYRNHSI FLADINQERGINESYKKNLMALKKFVMVKFLNDSIVDPVDSEWFGFYRSG QAKETIPLQETSLYTQDRLGLKEMDNAGQLVFLATEGDHLQLSEEWFYAH IIPFLG
    • Molecular weight60 kDa including tags
    • Amino acids1 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

  • FormLiquid
  • Additional notesProtein 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
  • FunctionRemoves 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 diseaseDefects 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 similaritiesBelongs to the palmitoyl-protein thioesterase family.
  • Cellular localizationLysosome.
  • Information by UniProt

Recombinant Human PPT1 protein images

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

References for Recombinant Human PPT1 protein (ab116764)

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"