Palmitoyl protein thioesterase 1 (PPT1) removes palmitate from specific cysteine residues in peptides and proteins. We have previously shown that a palmitoylated myelin glycoprotein. Po octapeptide (IRYCWLRR) can be specifically depalmitoylated by PPT1 in vitro (Cho and Dawson [1998] J. Neurochem. 1
Retina in various animal models of neuronal ceroid-lipofuscinosis
โ Scribed by Goebel, Hans H.
- Publisher
- John Wiley and Sons
- Year
- 1992
- Tongue
- English
- Weight
- 445 KB
- Volume
- 42
- Category
- Article
- ISSN
- 0148-7299
No coin nor oath required. For personal study only.
๐ SIMILAR VOLUMES
Five patients with juvenile neuronal ceroid-lipofuscinosis, who were shown to have absent or reduced serum prebetalipoprotein and a deficiency of polyunsaturated fatty acids in erythrocyte membrane lipids, were treated for a period of one year with supplements of fish oil extract (rich in omega-3 fa
We report the exclusion of late infantile neuronal ceroid lipofuscinosis in a fetus by assay of tripeptidyl peptidase I activity and by mutational analysis in chorionic villi. This is the ยฎrst pregnancy at risk for LINCL to be monitored by enzyme assay. No morphological abnormalities were detected.
## Diagnosis of late-infantile neuronal ceroid lipofuscinosis: A new sensitive method to assay lysosomal pepstatin-insensitive proteinase activity in human and animal specimens by capillary electrophoresis Batten disease, or human late-infantile neuronal ceroid lipofuscinosis (LINCL) is a familiar