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Part Description

LP32455-5   Palmitoyl protein thioesterase
Palmitoyl protein thioesterases are enzymes (EC 3.1.2.22) that remove thioester-linked fatty acyl groups such as palmitate from modified cysteine residues in proteins or peptides during lysosomal degradation.

Neuronal ceroid lipofuscinoses (NCL) represent a group of encephalopathies that occur in 1 in 12,500 children. Mutations in the palmitoyl protein thioesterase gene causing infantile neuronal ceroid lipofuscinosis. The most common mutation results in intracellular accumulation of the polypeptide and undetectable enzyme activity in the brain. Direct sequencing of cDNAs derived from brain RNA of INCL patients has shown a mis-sense transversion of A to T at nucleotide position 364, which results in substitution of Trp for Arg at position 122 in the protein - Arg 122 is immediately adjacent to a lipase consensus sequence that contains the putative active site Ser of PPT. The occurrence of this and two other independent mutations in the PPT gene strongly suggests that defects in this gene cause INCL. Copyright Text is available under the Creative Commons Attribution/Share-Alike License. See http://creativecommons.org/licenses/by-sa/3.0/ for details. Source: Wikipedia, Wikipedia

LOINC Names Get Info

Fully-Specified Name
Palmitoyl protein thioesterase:CCnt:Pt:Fibroblasts:Qn:
Long Common Name
Palmitoyl protein thioesterase [Enzymatic activity/mass] in Fibroblast
Short Name
PPT Fib-cCnt
Display Name
Palmitoyl protein thioesterase (Fibroblast) [Catalytic activity/Mass]
Consumer Name Alpha Get Info
Palmitoyl protein thioesterase, Fibroblast

Part Model Get Info

  • Component
    Palmitoyl protein thioesterase
    LP32455-5
    • Analyte
      Palmitoyl protein thioesterase
      LP32455-5
      • Component Numerator
        Palmitoyl protein thioesterase
        LP32455-5
        • Component Numerator Core
          Palmitoyl protein thioesterase
          LP32455-5
        • Component Numerator Core Suffix
          NULL
           
      • Component Denominator
        NULL
         
        • Component Denominator Core
          NULL
           
        • Component Denominator Core Suffix
          NULL
           
    • Challenge
      NULL
       
    • Adjustment
      NULL
       
    • Count
      NULL
       
  • Property
    CCnt
    LP6790-2
  • Time
    Pt
    LP6960-1
  • System
    Fibroblasts
    LP7234-0
    • System Core
      Fibroblasts
      LP7234-0
    • Super System
      NULL
       
  • Scale
    Qn
    LP7753-9
  • Method
    NULL
     

Basic Attributes

Class
CHEM
Type
Laboratory
First Released
Version 2.46
Last Updated
Version 2.46 (MIN)
Order vs. Observation
Both

Member of these Panels

LOINCLong Common Name
93703-7Palmitoyl protein thioesterase 1 and Tripeptidyl peptidase 1 panel - Fibroblast

Language Variants Get Info

TagLanguageTranslation
cs-CZCzech (Czechia)Palmitoyl-protein-thioesteráza:Katalytický obsah:Časový bod:Fibroblast:Kvantitativní:
de-ATGerman (Austria)Synonyms: Palmitoyl-Protein-Thioesterase /Fibroblasten
el-GRGreek (Greece)Θειοεστεράση πρωτεϊνών παλμιτοϋλίου:CCnt:Pt:Ινοβλάστες:Qn:
Synonyms: CCnt CHEM Pt Qn Θειοεστεράση πρωτεϊνών παλμιτοϋλίου Ινοβλάστες Πρωτεΐνη
es-ESSpanish (Spain)Palmitoil-protein-tioesterasa:Contenido catalítico:Punto temporal:Fibroblastos:Qn:
Synonyms: Cuantitativo
es-MXSpanish (Mexico)Palmitoil proteína tioesterasa:Contenido catalítico:Punto temporal:Fibroblasto:Cuantitativo:
fr-CAFrench (Canada)Palmitoyl-protéine thioestérase:Contenu catalytique:Temps ponctuel:Fibroblastes:Quantitatif:
fr-FRFrench (France)Palmitoyl protéine thioestérase:Catalytique/Masse:Ponctuel:Fibroblastes:Numérique:
fr-BEFrench (Belgium)Palmitoyl-protéine thioestérase:Contenu catalytique:Temps ponctuel:Fibroblastes:Quantitatif:
it-ITItalian (Italy)Palmitoil proteina tioesterasi:CCnt:Pt:Fibroblasti:Qn:
Synonyms: Chimica Contenuto Catalitico Fibroblasto Punto nel tempo (episodio)
nl-NLDutch (Netherlands)palmitoylproteïnthioesterase:enzymatische activiteit/massa:moment:fibroblast:kwantitatief:
pl-PLPolish (Poland)Tioesteraza białka palmitoilowego:zawartość katalityczna:punkt w czasie:fibroblasty:ilościowy:
Synonyms: Tioesteraza białka palmitylowego
ru-RURussian (Russian Federation)Пальмитоил протеин тиоэстераза:КаталСод:ТчкВрм:Фибробласты:Колич:
Synonyms: Белок Каталитическое содержание Количественный Точка во времени;Момент
tr-TRTurkish (Turkey)Palmitoil protein tiyoesteraz:Ktlİçrk:Zmlı:Fibroblastlar:Kant:
zh-CNChinese (China)棕榈酰蛋白硫酯酶:催化含量:时间点:成纤维细胞:定量型:
Synonyms: PPT;棕榈基硫酯酶;软酯酰蛋白硫酯酶 PR;Prot;成分 - 化学 - 蛋白质类;蛋白;朊;蛋白质类;蛋白质类物质 催化含量(单位质量);单位质量的酶单位数 化学;化学检验项目;化学检验项目类;化学类;化学试验;非刺激耐受型化学检验项目;非刺激耐受型化学检验项目类;非刺激耐受型化学试验;非刺激耐受型化学试验类 可用数量表示的;定量性;数值型;数量型;连续数值型标尺 时刻;随机;随意;瞬间 纤维原细胞;纤维母细胞

Example Units

UnitSource
nmol/h/mg{protein}Example UCUM Units

LOINC Terminology Service (API) using HL7® FHIR® Get Info

CodeSystem lookup
https://fhir.loinc.org/CodeSystem/$lookup?system=http://loinc.org&code=74365-8