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PPT1

  • Official Full Name

    palmitoyl-protein thioesterase 1

  • Overview

    The protein encoded by this gene is a small glycoprotein involved in the catabolism of lipid-modified proteins during lysosomal degradation. The encoded enzyme removes thioester-linked fatty acyl groups such as palmitate from cysteine residues. Defects in this gene are a cause of infantile neuronal ceroid lipofuscinosis 1 (CLN1, or INCL) and neuronal ceroid lipofuscinosis 4 (CLN4). Two transcript variants encoding different isoforms have been found for this gene.
  • Synonyms

    PPT1; palmitoyl-protein thioesterase 1; PPT; ceroid lipofuscinosis; neuronal 1; infantile; CLN1; INCL; PPT-1; palmitoyl-protein hydrolase 1; ceroid-palmitoyl-palmitoyl-protein thioesterase 1;

  • Recombinant Proteins
  • Cell & Tissue Lysates
  • Protein Pre-coupled Magnetic Beads
  • Arabidopsis thaliana (Mouse-ear cress)
  • Chicken
  • Cynomolgus Monkey
  • Human
  • Mouse
  • Oryza sativa subsp. japonica (Rice)
  • Rat
  • Rhesus Macaque
  • Zebrafish
  • E.coli
  • E.coli expression system
  • HEK293
  • Human Cell
  • In Vitro Cell Free System
  • Mammalian Cell
  • Mammalian cells
  • Wheat Germ
  • C
  • His
  • Fc
  • His (Fc)
  • Avi
  • N/A
Species Cat.# Product name Source (Host) Tag Protein Length Price
Human PPT1-29543TH Recombinant Human PPT1 Wheat Germ N/A 306 amino acids
Human PPT1-367H Recombinant Human PPT1, His-tagged Human Cell His Met1-Gly306
Human PPT1-366H Recombinant Human PPT1, Fc-tagged Human Cell Fc Met1-Gly306
Human PPT1-652H Recombinant Human PPT1 Protein, His-tagged E.coli His
Human PPT1-001HCL Recombinant Human PPT1 cell lysate Human Cell N/A
Human PPT1-3341H Recombinant Human PPT1 protein, His-tagged E.coli His 1-306 aa
Human PPT1-5970H Recombinant Human PPT1 Protein (Asp28-Gly306), C-His tagged Mammalian cells C-His Asp28-Gly306
Human PPT1-393HF Recombinant Full Length Human PPT1 Protein In Vitro Cell Free System 306 amino acids
Mouse PPT1-13281M Recombinant Mouse PPT1 Protein Mammalian Cell His
Mouse PPT1-7050M-B Recombinant Mouse PPT1 Protein Pre-coupled Magnetic Beads HEK293
Mouse PPT1-7050M Recombinant Mouse PPT1 Protein, His (Fc)-Avi-tagged HEK293 His (Fc)-Avi
Mouse Ppt1-534M Recombinant Mouse Ppt1 Protein, His-tagged E.coli His Asp28~Lys279
Rat PPT1-4647R Recombinant Rat PPT1 Protein Mammalian Cell His
Rat PPT1-4306R Recombinant Rat PPT1 Protein, His (Fc)-Avi-tagged HEK293 His (Fc)-Avi
Rat PPT1-4306R-B Recombinant Rat PPT1 Protein Pre-coupled Magnetic Beads HEK293
Arabidopsis thaliana (Mouse-ear cress) RFL17383AF Recombinant Full Length Arabidopsis Thaliana 4-Hydroxybenzoate Polyprenyltransferase, Mitochondrial(Ppt1) Protein, His-Tagged E.coli expression system His Full Length of Mature Protein (22-407)
Cynomolgus Monkey PPT1-808C Recombinant Cynomolgus PPT1 Protein, His-tagged Mammalian Cell His
Cynomolgus Monkey PPT1-551C Recombinant Cynomolgus Monkey PPT1 Protein, His (Fc)-Avi-tagged HEK293 His (Fc)-Avi
Cynomolgus Monkey PPT1-551C-B Recombinant Cynomolgus Monkey PPT1 Protein Pre-coupled Magnetic Beads HEK293
Oryza sativa subsp. japonica (Rice) RFL8989OF Recombinant Full Length Oryza Sativa Subsp. Japonica Phosphoenolpyruvate/Phosphate Translocator 1, Chloroplastic(Ppt1) Protein, His-Tagged E.coli expression system His Full Length of Mature Protein (67-408)
Rhesus Macaque PPT1-3581R Recombinant Rhesus monkey PPT1 Protein, His-tagged Mammalian Cell His
Rhesus Macaque PPT1-3399R Recombinant Rhesus Macaque PPT1 Protein, His (Fc)-Avi-tagged HEK293 His (Fc)-Avi
Rhesus Macaque PPT1-3399R-B Recombinant Rhesus Macaque PPT1 Protein Pre-coupled Magnetic Beads HEK293
Zebrafish PPT1-12524Z Recombinant Zebrafish PPT1 Mammalian Cell His
Chicken PPT1-2592C Recombinant Chicken PPT1 Mammalian Cell His
  • Involved Pathway
  • Protein Function
  • Interacting Protein
  • PPT1 Related Articles

PPT1 involved in several pathways and played different roles in them. We selected most pathways PPT1 participated on our site, such as Fatty acid elongation, Metabolic pathways, Fatty acid metabolism, which may be useful for your reference. Also, other proteins which involved in the same pathway with PPT1 were listed below. Creative BioMart supplied nearly all the proteins listed, you can search them on our site.

Pathway Name Pathway Related Protein
Fatty acid elongationPPT2;ELOVL4A;ELOVL4;HADHA;ELOVL7B;HADHAB;PPT1;HSD17B12A;ELOVL7
Metabolic pathwaysITPKC;OGDHA;GCDHB;MECR;PIGH;C1GALT1;AGK;DNMT3B;PMM2
Fatty acid metabolismACOX3;ACAA1;PPT1;ACAA1A;MECR;ACSL3B;EHHADH;ACADS;ACAT1
LysosomeCTSE;CTSC;GALCB;CTSLA;IDS;GBA;ATP6V0A1A;SLC11A2;CD68

PPT1 has several biochemical functions, for example, palmitoyl-(protein) hydrolase activity, palmitoyl-CoA hydrolase activity. Some of the functions are cooperated with other proteins, some of the functions could acted by PPT1 itself. We selected most functions PPT1 had, and list some proteins which have the same functions with PPT1. You can find most of the proteins on our site.

Function Related Protein
palmitoyl-(protein) hydrolase activityLYPLA2;LYPLA1;LYPLAL1;PPT2;PPT1
palmitoyl-CoA hydrolase activityACOT2;ACOT7;GNPAT;BAAT;ACOT8;ACOT1;ACOT5;THEM5;THEM4

PPT1 has direct interactions with proteins and molecules. Those interactions were detected by several methods such as yeast two hybrid, co-IP, pull-down and so on. We selected proteins and molecules interacted with PPT1 here. Most of them are supplied by our site. Hope this information will be useful for your research of PPT1.

GNAQ; ompR; pi3p; 15-deoxy-delta(12,14; MYC; cona_canen; 1C; VCAM1

Hu, J; Lu, JY; et al. Intravenous high-dose enzyme replacement therapy with recombinant palmitoyl-protein thioesterase reduces visceral lysosomal storage and modestly prolongs survival in a preclinical mouse model of infantile neuronal ceroid lipofuscinosis. MOLECULAR GENETICS AND METABOLISM 107:213-221(2012).
Chu-LaGraff, Q; Blanchette, C; et al. The Batten Disease Palmitoyl Protein Thioesterase 1 Gene Regulates Neural Specification and Axon Connectivity during Drosophila Embryonic Development. PLOS ONE 5:-(2010).
  • Q&As
  • Reviews

Q&As (5)

Ask a question
Can PPT1 mutations lead to other health problems beyond NCL? 03/21/2023

PPT1 mutations are primarily associated with NCL, but they can have varying effects on patients, and in some cases, they may present with atypical symptoms.

What medical condition is associated with mutations in the PPT1 gene? 07/29/2021

Mutations in the PPT1 gene are linked to Neuronal Ceroid Lipofuscinosis (NCL), a group of rare neurodegenerative disorders.

What are the clinical symptoms of NCL caused by PPT1 deficiency? 08/06/2020

Patients with PPT1-related NCL typically experience vision loss, seizures, cognitive decline, and motor dysfunction.

Is there a cure for NCL caused by PPT1 mutations? 09/30/2019

Currently, there is no cure for NCL. Treatment focuses on managing symptoms and improving the quality of life.

How does PPT1 deficiency lead to NCL? 03/19/2019

PPT1 deficiency results in the accumulation of lipofuscin, a fatty substance, in neurons, which causes neurodegeneration.

Customer Reviews (3)

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Reviews
11/20/2022

    It has been shown to play a crucial role in multiple cellular processes, making it highly valuable for investigations into cell signaling, inflammation, and immune responses.

    04/21/2020

      Incorporating the PPT1 protein into your research will not only meet but exceed your expectations, enabling you to advance your scientific discoveries with confidence.

      09/01/2017

        Researchers can leverage the unique properties of the PPT1 protein to gain insights into its biological functions and its impact on disease mechanisms.

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