Sialic acids. Enzymatic synthesis of Tay-Sachs ganglioside.
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چکیده
منابع مشابه
Preparation of radioactive Tay-Sachs ganglioside labeled in the sialic acid moiety.
A procedure is described for the preparation of Tay-Sachs ganglioside specifically labeled in the sialic acid portion of the molecule. Rat brain gangliosides were labeled biosynthetically by the intracranial injection of N-acetyl-(3)H-D-mannosamine. Radioactive gangliosides were isolated and selectively degraded with bacterial neuraminidase and rat liver beta-galactosidase to Tay-Sachs ganglios...
متن کاملThe catabolism of Tay-Sachs ganglioside in rat brain lysosomes.
The metabolism of Tay-Sachs ganglioside, Cer-Glc-Gal(NeuAc)-GalNAc (G& was investigated by using GNIZ specifically labeled with 3H in the NeuAc moiety or with W in GalNAc. There are two possible pathways for the catabolism of GM2 in brain, initiated via GMM-sialidase or GM2hexosaminidase. The products of the sialidase reaction were identified and are Cer-Glc-Gal-GalNAc and NeuAc; the hexosamini...
متن کاملThe metabolism of Tay-Sachs ganglioside: catabolic studies with lysosomal enzymes from normal and Tay-Sachs brain tissue.
The catabolism of Tay-Sachs ganglioside, N-acetylgalactosaminyl- (N-acetylneuraminosyl) -galactosylglucosylceramide, has been studied in lysosomal preparations from normal human brain and brain obtained at biopsy from Tay-Sachs patients. Utilizing Tay-Sachs ganglioside labeled with (14)C in the N-acetylgalactosaminyl portion or (3)H in the N-acetylneuraminosyl portion, the catabolism of Tay-Sac...
متن کاملTay-Sachs Disease
In 1881 British ophthalmologist Warren Tay made an unusual observation. He reported a cherry-red spot on the retina of a one-year-old patient, a patient who was also showing signs of progressive degeneration of the central nervous system [4] as manifested in the child?s physical and mental retardation [5]. This cherry-red spot is a characteristic that would eventually come to be associated with...
متن کاملLate-onset Tay-Sachs disease.
We discuss the assessment and differential diagnoses of a young adult Hungarian man with a 1-year history of a progressive and symmetric amyotrophic lateral sclerosis-like syndrome, along with irregular action tremor and stimulus-sensitive myoclonus of the arms. MR scan of the brain showed isolated cerebellar atrophy and formal neuropsychometric testing identified significant subclinical defici...
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ژورنال
عنوان ژورنال: Journal of Biological Chemistry
سال: 1975
ISSN: 0021-9258
DOI: 10.1016/s0021-9258(19)40993-9