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Results 1 - 6 of 6
EC Number
Application
Commentary
Reference
medicine
in vitro enzyme assay that can be readily used for the clinical diagnosis of both infantile neuronal ceroid lipofuscinosis patients and carrier
medicine
palmitoyl protein thioesterase 1, expressed in the human saliva is a reliable and non-invasive source for the diagnosis of infantile neuronal ceroid lipofuscinosis
medicine
if patients are diagnosed with any type of mental and/or physical retardation accompanied by strong and severe visual impairment, then the case physician may consider the possibility of infantile neuronal ceroid lipofuscinosis and conduct an examination of PPT activity level
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
medicine
homozygous palmitoyl-protein thioesterase PPT1 knockout mice reproduce the known features of infantile neuronal ceroid lipofuscinosis, developing signs of motor dysfunction at 5 months of age and death by around 8 months. PPT1 knockout mice treated with purified recombinant PPT1 corresponding to 12 mg/kg or 180 U/kg for a 25 g mouse administered intravenously weekly either from birth or beginning at 8 weeks of age surprisingly well tolerate the treatment and neither anaphylaxis nor antibody formation is observed. In mice treated from birth, survival increases from 236 to 271 days and the onset of motor deterioration is similarly delayed. In mice treated beginning at 8 weeks, no increases in survival or motor performance are seen. Outside the central nervous system, substantial clearance of autofluorescent storage material in many tissues is observed. Macrophages in spleen, liver and intestine are especially markedly improved, as are acinar cells of the pancreas and tubular cells of the kidney
nutrition
the litter sizes for females mated to obese males are significantly lower as compared to females mated with normal-diet-fed controls. Their serum high-density lipoprotein, low-density lipoprotein, cholesterol, and estradiol levels increase in obese males, but testosterone and follicle-stimulating hormone levels decrease. Testicular morphology disruptions include Sertoli-cell atrophy, disrupted tight junctions, and mitochondrial degeneration in spermatogenic cells. In rats fed a high-fat diet, palmitoyl-protein thioesterase PPT1 is upregulated. In a Sertoli-cell line cultured in a high-fat supplemented medium, PPT1 abundance Is accompanied by increases in the endocytic vesicle-associated protein, clathrin, and decreases in the tight junctional proteins, ZO-1 and occludin
Results 1 - 6 of 6