PPT1
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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
- Human
- Mouse
- Zebrafish
- Chicken
- Rhesus macaque
- Rat
- Cynomolgus
- Bovine
- Arabidopsis thaliana
- Rice
- Human Cells
- E.coli
- Mammalian Cells
- Wheat Germ
- HEK293
- In Vitro Cell Free System
- His
- GST
- Fc
- Non
- Avi
- Myc
Involved Pathway
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 |
|---|---|
| Lysosome | CTSSB.1,SCARB2,CTSE,ATP6V0C,ASAH1A,LGMN,AP1M1,FUCA1,ACP5A,GALCA |
| Fatty acid metabolism | ACADL,HADH,ACOX1,ACSL1,ACSL1A,ACAT1,HADHAA,MCAT,HSD17B12,SCDB |
| Fatty acid elongation | ELOVL5,ELOVL2,HSD17B12A,ELOVL7,ELOVL7A,TECR,ELOVL1B,HADHA,TECRB,ACOT2 |
| Metabolic pathways | NT5C1AA,MTMR8,TK1,CEL.1,HSD17B6,ATP6V0A2B,ASMT,CYP51A1,TM7SF2,MOGAT3 |
Protein Function
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-CoA hydrolase activity | THEM4,ACOT5,BAAT,ACOT1,ACOT8,THEM5,ACOT7,ACOT2,GNPAT |
| palmitoyl-(protein) hydrolase activity | PPT2,LYPLA2,LYPLA1,LYPLAL1 |
Interacting Protein
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
Resources
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References
- 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).
