Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Target Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Query: UMLS:C0205700 (
ash
)
15,125
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
Glycolipid biosynthesis in rat bone marrow cells has been studied with reference to four kinds of glycosyltransferases catalyzing the transfer of N-acetylgalactosamine, galactose, N-acetylneuraminic acid, and fucose to each glycolipid acceptor. It was demonstrated that glycosyltransferase activities which synthesize galactosylglucosylceramide (CDH) from glucosylceramide (
CMH
), N-acetylgalactosaminylgalactosylglucosylceramide (
GA2
) from CDH, galactosyl-N-acetylgalactosaminylgalactosylglucosylceramide (GA1) from
GA2
and N-acetylneuraminylgalactosyl-N-acetylgalactosaminylgalactosylglucosylceramide (Gm1b) from GA1 were all present in rat bone marrow cell homogenate. Fucosyltransferase activity catalyzing the transfer of fucose from GDP-fucose to GA1 was also recognized in the cell homogenate. Neutral glycolipid extracted from rat bone marrow cells was analyzed by thin layer chromatography and glycosidase treatments. The presence of glycolipids corresponding to
GA2
, GA1 and fucolipid was demonstrated. From these results, it was concluded that the biosynthesis of glycolipid through asialogangliosides is a major biosynthetic route in rat bone marrow cells.
...
PMID:Glycosphingolipid biosynthesis through asialogangliosides in rat bone marrow cells. 680 14
Mouse models for urea cycle disorders have been available for the past 30 y; however, until now, no measurements of urea production in vivo have been conducted. Urea entry rate was determined in Otc(spf-
ash
) and littermate controls employing a primed-continuous infusion of 15N15N urea. A saline infusion control, a complete mixture of amino acids (AA), or a glycine-alanine (GA) mixture was infused at 86 (AA1 and GA1) and 172 mg N.kg(-1).h(-1) (AA2 and
GA2
) to impose a defined nitrogen load on the urea cycle. Urea entry rate and plasma urea concentration increased (P < 0.001) as a consequence of the increase in the infusion rate of the complete mixture of amino acids, but the 2 genotypes did not differ (P = 0.96 and P = 0.44, respectively). The infusion of the GA mixture, however, decreased (P < 0.001) the plasma urea concentration and urea entry rate in Otc(spf-
ash
) mice compared with controls. At the highest level (
GA2
), urea entry rate was further depressed (P < 0.001), Otc(spf-
ash
) mice became hyperammonemic (1701 +/- 150 micromol/L), and hyperammonemic symptoms were evident. An acute hepatic enlargement (P < 0.001) was also evident in Otc(spf-
ash
) mice infused with
GA2
. These results show that despite vestigial OTC activity, Otc(spf-
ash
) mice were able to maintain ureagenesis at the same rate of control animals when a complete mixture of amino acids was infused. This implies that Otc(spf-
ash
) mice are able to dispose of ammonia, without apparent adverse effects, when a balance mixture of amino acids is provided, despite reduced enzyme activity.
...
PMID:Reduced ornithine transcarbamylase activity does not impair ureagenesis in Otc(spf-ash) mice. 1654 67
