PPT1
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Official Full Name
palmitoyl-protein thioesterase 1
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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
- 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 |
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Fatty acid elongation | PPT2;ELOVL4A;ELOVL4;HADHA;ELOVL7B;HADHAB;PPT1;HSD17B12A;ELOVL7 |
Metabolic pathways | ITPKC;OGDHA;GCDHB;MECR;PIGH;C1GALT1;AGK;DNMT3B;PMM2 |
Fatty acid metabolism | ACOX3;ACAA1;PPT1;ACAA1A;MECR;ACSL3B;EHHADH;ACADS;ACAT1 |
Lysosome | CTSE;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 |
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palmitoyl-(protein) hydrolase activity | LYPLA2;LYPLA1;LYPLAL1;PPT2;PPT1 |
palmitoyl-CoA hydrolase activity | ACOT2;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
- Q&As
- Reviews
Q&As (5)
Ask a questionPPT1 mutations are primarily associated with NCL, but they can have varying effects on patients, and in some cases, they may present with atypical symptoms.
Mutations in the PPT1 gene are linked to Neuronal Ceroid Lipofuscinosis (NCL), a group of rare neurodegenerative disorders.
Patients with PPT1-related NCL typically experience vision loss, seizures, cognitive decline, and motor dysfunction.
Currently, there is no cure for NCL. Treatment focuses on managing symptoms and improving the quality of life.
PPT1 deficiency results in the accumulation of lipofuscin, a fatty substance, in neurons, which causes neurodegeneration.
Customer Reviews (3)
Write a reviewIt 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.
Incorporating the PPT1 protein into your research will not only meet but exceed your expectations, enabling you to advance your scientific discoveries with confidence.
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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