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Family Report for: Palmitoyl-protein_thioesterase


Block X
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. 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. PPT2 removes thioester-linked fatty acyl groups from various substrates including S-palmitoyl-CoA. PPT1 and -2 perform non-redundant roles in lysosomal thioester catabolism. Neuronal ceroid lipofuscinoses (NCL) represent a group of encephalopathies that occur in 1 in 12,500 children. Mutations in the palmitoyl protein thioesterase gene (human-PPT1) causing infantile neuronal ceroid lipofuscinosis. The most common mutation results in intracellular accumulation of the polypeptide and undetectable enzyme activity in the brain. Insensitive to commonly used serine-modifying reagents phenylmethylsulfonyl fluoride (PMSF) and diisopropylfluorophosphate. See paper of structure of bovine enzyme 1EXW (Das et al. 2000). PPT2 deficiency in a recent transgenic mouse model is associated with a form of neuronal ceroid lipofuscinosis, suggesting that PPT1 and -2 perform non-redundant roles in lysosomal thioester catabolism. Peptide based reversible inhibitors may have a role as chaperone that could facilitate the transport of mutant PPT1 variants to the lysosome where improved enzyme stability may be observed in the lower pH environment Family TE20 in ThYme database
1 Disease(s)

IPR002472 (Palmitoyl protein thioesterase), IPR030295 (Lysosomal thioesterase PPT2), IPR030294 (Palmitoyl-protein thioesterase 1)
PF02089 (Palm_thioest)

Peptide in
Nucleotide in
Alignment with Multalin
|Text only/graphic display
Seed alignment with MAFFT
|No colour/coloured with Mview
Alignment with MAFFT
|No colour/coloured with Mview
|Graphical display, obtained with the dnd file produced by Clustalw

14 more
    Title: Depalmitoylation by Palmitoyl-Protein Thioesterase 1 in Neuronal Health and Degeneration
    Koster KP, Yoshii A
    Ref: Front Synaptic Neurosci, 11:25, 2019 : PubMed


    Title: Palmitoyl:protein thioesterase (PPT1) inhibitors can act as pharmacological chaperones in infantile Batten disease
    Dawson G, Schroeder C, Dawson PE
    Ref: Biochemical & Biophysical Research Communications, 395:66, 2010 : PubMed


    Title: Didemnin binds to the protein palmitoyl thioesterase responsible for infantile neuronal ceroid lipofuscinosis
    Crews CM, Lane WS, Schreiber SL
    Ref: Proc Natl Acad Sci U S A, 93:4316, 1996 : PubMed


Other Papers

No structure scheme yet for this family

Structures in Palmitoyl-protein_thioesterase family (5)

Genes Proteins in Palmitoyl-protein_thioesterase family (59)

Fragments of genes in Palmitoyl-protein_thioesterase family (13)

Substrates of some enzymes in the Palmitoyl-protein_thioesterase family (1)

Inhibitors of some enzymes in the Palmitoyl-protein_thioesterase family (7)

Send your questions or comments to :
Mail to: Nicolas Lenfant, Thierry Hotelier, Yves Bourne, Pascale Marchot and Arnaud Chatonnet.
Please cite: Lenfant 2013 Nucleic.Acids.Res. or Marchot Chatonnet 2012 Prot.Pept Lett.
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