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6  structures 335  species 1  interaction 683  sequences 14  architectures

Family: Palm_thioest (PF02089)

Summary: Palmitoyl protein thioesterase

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This is the Wikipedia entry entitled "Palmitoyl protein thioesterase". More...

Palmitoyl protein thioesterase Edit Wikipedia article

Palmitoyl protein thioesterase
1eh5 opm.png
Palmitoyl protein thioesterase 1. Red plane shows hydrocarbon boundary of the lipid bilayer
Symbol Palm_thioest
Pfam PF02089
Pfam clan CL0028
InterPro IPR002472
SCOP 1exw
OPM superfamily 135
OPM protein 1eh5
palmitoyl [protein] hydrolase
EC number
CAS number 150605-49-5
IntEnz IntEnz view
ExPASy NiceZyme view
MetaCyc metabolic pathway
PRIAM profile
PDB structures RCSB PDB PDBe PDBsum
Gene Ontology AmiGO / EGO

Palmitoyl protein thioesterases are enzymes (EC 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.[1] 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.


Human proteins containing this domain include:

palmitoyl-protein thioesterase 1
Symbol PPT1
Alt. symbols PPT
Entrez 5538
HUGO 9325
OMIM 600722
RefSeq NM_000310
UniProt P50897
Other data
EC number
Locus Chr. 1 p32
palmitoyl-protein thioesterase 2
Symbol PPT2
Alt. symbols G14
Entrez 9374
HUGO 9326
OMIM 603298
RefSeq NM_138717
UniProt Q9UMR5
Other data
EC number
Locus Chr. 6 p21.3

See also


  1. ^ Hofmann SL, Vesa J, Hellsten E, Verkruyse LA, Camp LA, Rapola J, Santavuori P, Peltonen L (1995). "Mutations in the palmitoyl protein thioesterase gene causing infantile neuronal ceroid lipofuscinosis". Nature 376 (6541): 584–587. doi:10.1038/376584a0. PMID 7637805. 

External links

This article incorporates text from the public domain Pfam and InterPro IPR002472

This page is based on a Wikipedia article. The text is available under the Creative Commons Attribution/Share-Alike License.

This tab holds the annotation information that is stored in the Pfam database. As we move to using Wikipedia as our main source of annotation, the contents of this tab will be gradually replaced by the Wikipedia tab.

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Internal database links

External database links

This tab holds annotation information from the InterPro database.

InterPro entry IPR002472

This entry represents the palmitoyl protein thioesterase family. Its members include palmitoyl-protein thioesterase 1 and lysosomal thioesterase 2 (PPT1 and PPT2). PPT1 is responsible for the removal of a palmitate group from its substrate proteins, which may include presynaptic proteins like SNAP-25, cysteine string protein (CSP), dynamin, and synaptotagmin [PUBMED:24091420]. PPT2 removes thioester-linked fatty acyl groups from various substrates including S-palmitoyl-CoA [PUBMED:12855696]. PPT1 and -2 perform non-redundant roles in lysosomal thioester catabolism [PUBMED:12855696].

Mutations in the PPT1 gene cause infantile-onset neuronal ceroid lipofuscinosis (INCL), which is a severe pediatric neurodegenerative disorder [PUBMED:23747979].

This entry also includes uncharacterised proteins from fungi and plants.

Gene Ontology

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Domain organisation

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Seed source: IPR002472
Previous IDs: none
Type: Domain
Author: Mian N, Bateman A
Number in seed: 1
Number in full: 683
Average length of the domain: 232.30 aa
Average identity of full alignment: 31 %
Average coverage of the sequence by the domain: 81.25 %

HMM information View help on HMM parameters

HMM build commands:
build method: hmmbuild -o /dev/null HMM SEED
search method: hmmsearch -Z 11927849 -E 1000 --cpu 4 HMM pfamseq
Model details:
Parameter Sequence Domain
Gathering cut-off 21.1 21.1
Trusted cut-off 21.2 21.2
Noise cut-off 21.0 21.0
Model length: 279
Family (HMM) version: 12
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Species distribution

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For those sequences which have a structure in the Protein DataBank, we use the mapping between UniProt, PDB and Pfam coordinate systems from the PDBe group, to allow us to map Pfam domains onto UniProt sequences and three-dimensional protein structures. The table below shows the structures on which the Palm_thioest domain has been found. There are 6 instances of this domain found in the PDB. Note that there may be multiple copies of the domain in a single PDB structure, since many structures contain multiple copies of the same protein seqence.

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