Enzymatic deficiency in neurological mutants. Brain uridine diphosphate galactose: Ceramide galactosyl transferase in jimpy mouse
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چکیده
منابع مشابه
Generalised uridine diphosphate galactose-4-epimerase deficiency.
The generalised form of epimerase deficiency galactosaemia has been described in only two children from unrelated families. Their progress is reported and three other affected children from these families are described. The initial presentation was similar to classic galactosaemia. Despite treatment all have shown poor growth and moderate learning difficulties. Three have sensorineural deafness...
متن کاملFrog brain uridine diphosphate galactose-N-acetylgalactosaminyl-N-acetylneuraminylgalactosylglucosylceramide galactosyltransferase.
1. The enzyme that catalyses the transfer of galactose from UDP-galactose to N-acetylgalactosaminyl-(1-->4)-N-acetylneuraminyl-(2-->3)-galactosyl-(1-->4)-glucosylceramide (G(M2)) was found mainly in the heavy- and light-microsomal fractions of the adult frog brain. 2. The subcellular distribution of the enzyme, UDP-galactose-G(M2) galactosyltransferase, parallels that of gangliosides in adult f...
متن کاملEnzymatic Synthesis of Uridine Diphosphate Xylose and Uridine Diphosphate Arabinose.
It has been shown previously' that mung bean seedlings contain a mixture of sugar nucleotides consisting of UDPG,2 UDPGal, UDPXy, and UDPAr. In the present communication evidence is presented that these bean seedlings contain an enzymatic system capable of catalyzing the reversible formation of UDPXy and UDPAr from UTP and a-D-Xy-l-P. When a-D-Xy-l-P and UTP were incubated in the presence of an...
متن کاملThe Intermediate in the Uridine Diphosphate Galactose P-Epimerase Reaction
Incubation of UDP-galactose with substrate amounts of UDP-galactose 4-epimerase (EC 5.1.3.2) from Escherichia coli for different times between 2 and 120 min, followed by reduction with NaB3H4 invariably yields a mixture of labeled UDP-glucose and UDP-galactose. Chemical degradation of tritiated hexoses, isolated after NsB3H4 reduction of 2-min incubation mixtures, shows all of the tritium assoc...
متن کاملThe Intermediate in the Uridine Diphosphate Galactose P-Epimerase Reaction
Incubation of UDP-galactose with substrate amounts of UDP-galactose 4-epimerase (EC 5.1.3.2) from Escherichia coli for different times between 2 and 120 min, followed by reduction with NaB3H4 invariably yields a mixture of labeled UDP-glucose and UDP-galactose. Chemical degradation of tritiated hexoses, isolated after NsB3H4 reduction of 2-min incubation mixtures, shows all of the tritium assoc...
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ژورنال
عنوان ژورنال: FEBS Letters
سال: 1970
ISSN: 0014-5793
DOI: 10.1016/0014-5793(70)80267-8