Recombinant人PPT1 protein (ab116764)

概述

描述

  • 性质Recombinant
  • 来源Wheat germ
  • 氨基酸序列
    • AccessionP50897
    • 种属Human
    • 序列MASPGCLWLLAVALLPWTCASRALQHLDPPAPLPLVIWHGMGDSCCNPLS MGAIKKMVEKKIPGIYVLSLEIGKTLMEDVENSFFLNVNSQVTTVCQALA KDPKLQQGYNAMGFSQGGQFLRAVAQRCPSPPMINLISVGGQHQGVFGLP RCPGESSHICDFIRKTLNAGAYSKVVQERLVQAEYWHDPIKEDVYRNHSI FLADINQERGINESYKKNLMALKKFVMVKFLNDSIVDPVDSEWFGFYRSG QAKETIPLQETSLYTQDRLGLKEMDNAGQLVFLATEGDHLQLSEEWFYAH IIPFLG
    • 分子量60 kDa including tags
    • 氨基酸1 to 306

技术指标

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.

  • 应用

    ELISA

    SDS-PAGE

    Western blot

  • 形式Liquid
  • 补充说明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...

制备和贮存

  • 稳定性和存储

    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

常规信息

  • 别名
    • 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
  • 功能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.
  • 疾病相关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).
  • 序列相似性Belongs to the palmitoyl-protein thioesterase family.
  • 细胞定位Lysosome.
  • Information by UniProt

Recombinant Human PPT1 protein 图像

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

Recombinant Human PPT1 protein (ab116764)参考文献

ab116764 has not yet been referenced specifically in any publications.

Product Wall

There are currently no Abreviews or Questions for ab116764.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"