Palmitoyl-protein thioesterase 1

Details

Name
Palmitoyl-protein thioesterase 1
Synonyms
  • 3.1.2.22
  • Palmitoyl-protein hydrolase 1
  • PPT
  • PPT-1
Gene Name
PPT1
Organism
Humans
Amino acid sequence
>lcl|BSEQ0009459|Palmitoyl-protein thioesterase 1
MASPGCLWLLAVALLPWTCASRALQHLDPPAPLPLVIWHGMGDSCCNPLSMGAIKKMVEK
KIPGIYVLSLEIGKTLMEDVENSFFLNVNSQVTTVCQALAKDPKLQQGYNAMGFSQGGQF
LRAVAQRCPSPPMINLISVGGQHQGVFGLPRCPGESSHICDFIRKTLNAGAYSKVVQERL
VQAEYWHDPIKEDVYRNHSIFLADINQERGINESYKKNLMALKKFVMVKFLNDSIVDPVD
SEWFGFYRSGQAKETIPLQETSLYTQDRLGLKEMDNAGQLVFLATEGDHLQLSEEWFYAH
IIPFLG
Number of residues
306
Molecular Weight
34193.245
Theoretical pI
Not Available
GO Classification
Functions
palmitoyl-(protein) hydrolase activity / palmitoyl-CoA hydrolase activity
Processes
adult locomotory behavior / associative learning / brain development / cellular lipid metabolic process / cellular protein catabolic process / cofactor metabolic process / cofactor transport / grooming behavior / lipid catabolic process / long-chain fatty-acyl-CoA biosynthetic process / lysosomal lumen acidification / membrane raft organization / negative regulation of apoptotic process / negative regulation of cell growth / negative regulation of neuron apoptotic process / nervous system development / neuron development / neurotransmitter secretion / pinocytosis / positive regulation of pinocytosis / positive regulation of receptor-mediated endocytosis / protein catabolic process / protein depalmitoylation / protein transport / receptor-mediated endocytosis / regulation of phospholipase A2 activity / regulation of synapse structure or activity / response to stimulus / small molecule metabolic process / sphingolipid catabolic process / triglyceride biosynthetic process / visual perception
Components
axon / cytosol / dendrite / extracellular exosome / extracellular region / extracellular space / Golgi apparatus / lysosomal lumen / lysosome / membrane / membrane raft / neuronal cell body / nucleus / synaptic vesicle
General Function
Palmitoyl-coa hydrolase activity
Specific Function
Removes thioester-linked fatty acyl groups such as palmitate from modified cysteine residues in proteins or peptides during lysosomal degradation. Prefers acyl chain lengths of 14 to 18 carbons.
Pfam Domain Function
Transmembrane Regions
Not Available
Cellular Location
Lysosome
Gene sequence
>lcl|BSEQ0021878|Palmitoyl-protein thioesterase 1 (PPT1)
ATGGCGTCGCCCGGCTGCCTGTGGCTCTTGGCTGTGGCTCTCCTGCCATGGACCTGCGCT
TCTCGGGCGCTGCAGCATCTGGACCCGCCGGCGCCGCTGCCGTTGGTGATCTGGCATGGG
ATGGGAGACAGCTGTTGCAATCCCTTAAGCATGGGTGCTATTAAAAAAATGGTGGAGAAG
AAAATACCTGGAATTTACGTCTTATCTTTAGAGATTGGGAAGACCCTGATGGAGGACGTG
GAGAACAGCTTCTTCTTGAATGTCAATTCCCAAGTAACAACAGTGTGTCAGGCACTTGCT
AAGGATCCTAAATTGCAGCAAGGCTACAATGCTATGGGATTCTCCCAGGGAGGCCAATTT
CTGAGGGCAGTGGCTCAGAGATGCCCTTCACCTCCCATGATCAATCTGATCTCGGTTGGG
GGACAACATCAAGGTGTTTTTGGACTCCCTCGATGCCCAGGAGAGAGCTCTCACATCTGT
GACTTCATCCGAAAAACACTGAATGCTGGGGCGTACTCCAAAGTTGTTCAGGAACGCCTC
GTGCAAGCCGAATACTGGCATGACCCCATAAAGGAGGATGTGTATCGCAACCACAGCATC
TTCTTGGCAGATATAAATCAGGAGCGGGGTATCAATGAGTCCTACAAGAAAAACCTGATG
GCCCTGAAGAAGTTTGTGATGGTGAAATTCCTCAATGATTCCATTGTGGACCCTGTAGAT
TCGGAGTGGTTTGGATTTTACAGAAGTGGCCAAGCCAAGGAAACCATTCCCTTACAGGAG
ACCTCCCTGTACACACAGGACCGCCTGGGGCTAAAGGAAATGGACAATGCAGGACAGCTA
GTGTTTCTGGCTACAGAAGGGGACCATCTTCAGTTGTCTGAAGAATGGTTTTATGCCCAC
ATCATACCATTCCTTGGATGA
Chromosome Location
1
Locus
Not Available
External Identifiers
ResourceLink
UniProtKB IDP50897
UniProtKB Entry NamePPT1_HUMAN
HGNC IDHGNC:9325
General References
  1. Vesa J, Hellsten E, Verkruyse LA, Camp LA, Rapola J, Santavuori P, Hofmann SL, Peltonen L: Mutations in the palmitoyl protein thioesterase gene causing infantile neuronal ceroid lipofuscinosis. Nature. 1995 Aug 17;376(6541):584-7. [Article]
  2. Crews CM, Lane WS, Schreiber SL: Didemnin binds to the protein palmitoyl thioesterase responsible for infantile neuronal ceroid lipofuscinosis. Proc Natl Acad Sci U S A. 1996 Apr 30;93(9):4316-9. [Article]
  3. Schriner JE, Yi W, Hofmann SL: cDNA and genomic cloning of human palmitoyl-protein thioesterase (PPT), the enzyme defective in infantile neuronal ceroid lipofuscinosis. Genomics. 1996 Jun 15;34(3):317-22. [Article]
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  7. Lu JY, Verkruyse LA, Hofmann SL: Lipid thioesters derived from acylated proteins accumulate in infantile neuronal ceroid lipofuscinosis: correction of the defect in lymphoblasts by recombinant palmitoyl-protein thioesterase. Proc Natl Acad Sci U S A. 1996 Sep 17;93(19):10046-50. [Article]
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  14. Das AK, Becerra CH, Yi W, Lu JY, Siakotos AN, Wisniewski KE, Hofmann SL: Molecular genetics of palmitoyl-protein thioesterase deficiency in the U.S. J Clin Invest. 1998 Jul 15;102(2):361-70. [Article]
  15. van Diggelen OP, Thobois S, Tilikete C, Zabot MT, Keulemans JL, van Bunderen PA, Taschner PE, Losekoot M, Voznyi YV: Adult neuronal ceroid lipofuscinosis with palmitoyl-protein thioesterase deficiency: first adult-onset patients of a childhood disease. Ann Neurol. 2001 Aug;50(2):269-72. [Article]
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Drug Relations

Drug Relations
DrugBank IDNameDrug groupPharmacological action?ActionsDetails
DB02035Hexadecanesulfonyl fluorideexperimentalunknownDetails
DB03796Palmitic AcidapprovedunknownDetails