Mannosidosis in angus cattle: Partial characterization of two mannose containing oligosaccharides

Enzyme Replacement Therapy—An Experiment of Nature in a Chimeric Mannosidosis Calf

“…Putrescine combines with a compound derived from L-methionine to form spermidine, which in turn yields spermine. O D C has been characterized in a variety of tissues, including fetal rat and fetal chick (16), and to a limited extent in human fetal brain and liver (17).…”

Glycopeptide storage in skin fibroblasts cultured from a patient with alpha-mannosidase deficiency.

“…Cell extracts were found to have a low specific activity with respect to this carbohydrate-peptide (GlcNAcAsn) cleaving enzyme when compared to solid tissues such as kidney or other cultured cell strains such as rat astrocytoma RGC-6 (Table V). Although some activity was detectable in vitro, the low level of activity may be related to the fact that the storage material in the cells is glycopeptide in nature whereas the urinary excretion material is mainly oligosaccharide (12)(13)(14)(15) 2 Chromatographic profiles of material isolated from a water-soluble extract of normal, mannosidosis heterozygote, Hurler's disease, and mannosidosis homozygote fibroblast cells. Cells (8 X 107) were grown in fresh modified minimal essential medium for 3 wk, harvested, frozen and thawed 3 times, and disrupted by sonication, and the 8,000 g supernatant solution was applied to a Sephadex G-50 (1.0 X 180 cm) column equilibrated with water.…”

“…In this study, normal human skin fibroblast a-mannosidase showed pH optima at 3.5-4.0 and 6.5. a-Mannosidase activity in fibroblasts from the proband, when assayed with 4-methylumbelliferyl-a-D-mannoside, was less than 1% of normal activity at pH 3.5. The peak of activity at pH 6.0, which may be equivalent to the a-mannosidase C localized to the Golgi in human liver (33) (13,36) in which a-mannosidase activity is greatly diminished, and the storage material (tetrasaccharide) apparently has the structure ManGlcNAc-Man-GlcNAc (13).…”

“…An equimolar ratio of mannose and N-acetylglucosamine was also found in a mixture of oligosaccharides isolated from the urine of a patient with Sandhoff's disease (11), but no structures were proposed. Further, pentasaccharide with the structure Man-Man-Man-Man-GlcNAc (together with a related tetrasaccharide and trisaccharide) has been purified from the urine of patients with mannosidosis by Norden et al (12)(13)(14) and in this laboratory (15). From our present knowledge, one can envision sialo- (25).…”

Deletion of the msdS/AfmsdC gene induces abnormal polarity and septation in Aspergillus fumigatus

“…DISCUSSION a-Mannosidase I is known to play an important role in the processing of mannose-containing glycans. In humans (Rose, 1967) and cattle (Burditt et al, 1978;Norden et al, 1973;Phillips et al, 1974), a deficiency in a-mannosidase can result in the lethal disease mannosidosis. In Drosophila melanogaster, deletion of the Golgi mannosidase I (MAS-1) is viable, and the null organisms synthesize the same range of oligosaccharides as the wild-type, albeit with different ratios (Roberts et al, 1998).…”