Abstract
Palmitoyl-protein thioesterase (PPT) is a lysosomal enzyme that removes fatty acids bound in thioester linkage to cysteine residues in fatty acylated proteins (1–4). Mutations in the PPT gene that affect enzyme activity cause a recessively inherited lysosomal storage disorder, infantile neuronal ceroid lipofuscinosis (INCL) (5). The disorder is characterized by severe central nervous system degeneration and lysosomal inclusions in the form of granular osmiophilic deposits in many tissues (6,7).
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References
Camp, L. A. and Hofmann, S. L. (1993) Purification and properties of a palmitoyl-protein thioesterase that cleaves palmitate from H-Ras. J. Biol. Chem. 268, 22,566–22,574.
Camp, L. A., Verkruyse, L. A., Afendis, S. J., Slaughter, C. A., and Hofmann, S. L. (1994) Molecular cloning and expression of palmitoyl-protein thioesterase. J. Biol. Chem. 269, 23,212–23,219.
Camp, L. A. and Hofmann, S. L. (1995) Assay and isolation of a palmitoyl-protein thioesterase from bovine brain using palmitoylated H-Ras as a substrate. Methods Enzymol. 250, 336–347.
Verkruyse, L. A. and Hofmann, S. L. (1996) Lysosomal targeting of palmitoyl-protein thioesterase. J. Biol. Chem. 271, 15,831–15,836.
Vesa, J., Hellsten, E., Verkruyse, L. A., Camp, L. A., Rapola, J., Santavuori, P., et al. (1995) Mutations in the palmitoyl protein thioesterase gene causing infantile neuronal ceroid lipofuscinosis. Nature 376, 584–587.
Haltia, M., Rapola, J., Santavuori, P., and Keranen, A. (1973) Infantile type of so-called neuronal ceroid-lipofuscinosis. Part 2. Morphological and biochemical studies. J. Neurol. Sci. 18, 269–285.
Haltia, M., Rapola, J., and Santavuori, P. (1973) Infantile type of so-called neuronal ceroid lipofuscinosis. Part I. Histological and electron microscopic studies. Acta Neuropath. 26, 157–170.
Lu, J.-Y., Verkruyse, L. A., and Hofmann, S. L. (1996) 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. USA 93, 10,046–10,050.
Soyombo, A. A. and Hofmann, S. L. (1997) Molecular cloning and expression of PPT2, a homolog of lysosomal palmitoyl-protein thioesterase with a distinct substrate specificity. J. Biol. Chem. 272, 27,456–27,463.
Juguelin, H. and Cassagne, C. (1984) Assay of long-chain acyl-CoAs in a complex reaction mixture. Anal. Biochem. 142, 329–335.
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Hofmann, S.L., Lu, JY. (1998). Metabolic Labeling of Protein-Derived Lipid Thioesters in Palmitoyl-Protein Thioesterase-Deficient Cells. In: Gelb, M.H. (eds) Protein Lipidation Protocols. Methods in Molecular Biology, vol 116. Humana Press. https://doi.org/10.1385/1-59259-264-3:213
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DOI: https://doi.org/10.1385/1-59259-264-3:213
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