Any feedback?
Please rate this page
(literature.php)
(0/150)

BRENDA support

Literature summary extracted from

  • Lu, J.Y.; Hu, J.; Hofmann, S.L.
    Human recombinant palmitoyl-protein thioesterase-1 (PPT1) for preclinical evaluation of enzyme replacement therapy for infantile neuronal ceroid lipofuscinosis (2010), Mol. Genet. Metab., 99, 374-378.
    View publication on PubMedView publication on EuropePMC

Application

EC Number Application Comment Organism
3.1.2.22 medicine enzyme may be useful as an adjunct to central nervous system-directed therapies and may be used as a starting point for modifications designed to improve brain delivery Homo sapiens

Cloned(Commentary)

EC Number Cloned (Comment) Organism
3.1.2.22 fragment corresponding to the entire coding region of PPT1 amplified from pCMV5-hPPT1, and cloned into XhoI sites in the polylinker region of expression vector pMSXND1, overexpressed in CHO cells. PPT1 injected intravenously into PPT1-deficient mice Homo sapiens

Organism

EC Number Organism UniProt Comment Textmining
3.1.2.22 Homo sapiens
-
-
-

Purification (Commentary)

EC Number Purification (Comment) Organism
3.1.2.22 by centrifugation, dialysis and gel filtration Homo sapiens

Source Tissue

EC Number Source Tissue Comment Organism Textmining
3.1.2.22 additional information lymphoblasts from patients with infantile neuronal ceroid lipofuscinosis, deficient of PPT1 Homo sapiens
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3.1.2.22 4-methylumbelliferyl 6-S-palmitoyl-6-thio-beta-D-glucoside + H2O
-
 Homo sapiens 4-methylumbelliferyl 6-thio-beta-D-glucoside + palmitate
-
 ?

Synonyms

EC Number Synonyms Comment Organism
3.1.2.22 palmitoyl-protein thioesterase-1
-
 Homo sapiens
3.1.2.22 PPT1
-
 Homo sapiens

General Information

EC Number General Information Comment Organism
3.1.2.22 physiological function when injected intravenously into PPT1-deficient mice, the clearance of recombinant PPT1 from plasma is rapid, with a half-life of 10 min. Most of the injected dose is distributed to the kidney and liver and potentially corrective levels are also observed in heart, lung and spleen. Brain uptake is minimal. The enzyme is largely mannose 6-phosphorylated and takes up rapidly by PPT1-deficient immortalized lymphoblasts derived from infantile neuronal ceroid lipofuscinosis patients Homo sapiens