PPT2 removes thioester-linked fatty acyl groups from various substrates including S-palmitoyl-CoA []. PPT2 deficiency in mice manifests as a neurodegenerative disorder with visceral features [].
Ppt2 is a phosphopantetheine:protein transferase (PPTase) that transfers the 4'-phosphopantetheine moiety from coenzyme A to a Ser of mitochondrial acyl-carrier-protein [].
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 []. 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 [].Mutations in the PPT1 gene cause infantile-onset neuronal ceroid lipofuscinosis (INCL), which is a severe pediatric neurodegenerative disorder [].This entry also includes uncharacterised proteins from fungi and plants.
