概述

描述

  • 性质
    Recombinant
  • 来源
    Wheat germ
  • 氨基酸序列
    • 种属
      Human
    • 序列
      MGLQACLLGLFALILSGKCSYSPEPDQRRTLPPGWVSLGRADPEEELSLT FALRQQNVERLSELVQAVSDPSSPQYGKYLTLENVADLVRPSPLTLHTVQ KWLLAAGAQKCHSVITQDFLTCWLSIRQAELLLPGAEFHHYVGGPTETHV VRSPHPYQLPQALAPHVDFVGGLHRFPPTSSLRQRPEPQVTGTVGLHLGV TPSVIRKRYNLTSQDVGSGTSNNSQACAQFLEQYFHDSDLAQFMRLFGGN FAHQASVARVVGQQGRGRAGIEASLDVQYLMSAGANISTWVYSSPGRHEG QEPFLQWLMLLSNESALPHVHTVSYGDDEDSLSSAYIQRVNTELMKAAAR GLTLLFASGDSGAGCWSVSGRHQFRPTFPASSPYVTTVGGTSFQEPFLIT NEIVDYISGGGFSNVFPRPSYQEEAVTKFLSSSPHLPPSSYFNASGRAYP DVAALSDGYWVVSNRVPIPWVSGTSASTPVFGGILSLINEHRILSGRPPL GFLNPRLYQQHGAGLFDVTRGCHESCLDEEVEGQGFCSGPGWDPVTGWGT PNFPALLKTLLNP
    • 氨基酸
      1 to 563
    • 标签
      proprietary tag N-Terminus

技术指标

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.

  • 应用

    ELISA

    Western blot

  • 形式
    Liquid
  • 补充说明
    Protein concentration is above or equal to 0.05 mg/ml.
  • 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: 0.31% Glutathione, 0.79% Tris HCl

常规信息

  • 别名
    • 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
  • 功能
    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.
  • 组织特异性
    Detected in all tissues examined with highest levels in heart and placenta and relatively similar levels in other tissues.
  • 疾病相关
    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.
  • 序列相似性
    Belongs to the peptidase S53 family.
  • 翻译后修饰
    Activated by autocatalytic proteolytical processing upon acidification. N-glycosylation is required for processing and activity.
  • 细胞定位
    Lysosome. Melanosome. Identified by mass spectrometry in melanosome fractions from stage I to stage IV.
  • Information by UniProt

图片

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

文献

ab152286 has not yet been referenced specifically in any publications.

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