| 1. |
- Chester, Alan, et al.
(författare)
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Phenyl beta-D-Galactopyranoside as an Acceptor Substrate for the Blood-Group H Gene-Associated Guanosine Diphosphate L-Fucose:beta-D-Galactosyl alpha-2-L-Fucosyltransferase
- 1976
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Ingår i: European Journal of Biochemistry. - 0014-2956. ; 69:2, s. 583-592
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Tidskriftsartikel (refereegranskat)abstract
- Phenyl β-d-galactopyranoside was found to be an efficient acceptor of l-[14C]fucose when guanosine diphosphate l-[14C]fucose was used as the donor substrate and human serum, submaxillary glands or stomach mucosa were the sources of l-fucosyltransferase. The enzyme utilising phenyl β-D-galactoside was present in the serum of donors of all ABO blood-groups examined, except those of the rare Oh (Bombay) and Bh phenotypes, but was clearly demonstrable in submaxillary gland preparations only when the glands came from individuals who were secretors of ABH blood-group substances. This distribution coincides with that previously established for the blood-group H gene-specified α-2-l-fucosyltransferase. The product of l-[14C]fucosyl transfer to phenyl β-d-galactoside is separable from the other radioactive components in the enzyme digest by chromatography for 4 h in ethyl acetate/pyridine/water (10/4/3, by vol.); it was characterised as phenyl 2-O-(α-l-[14C]fucopyranosyl)β-d-galactopyranoside by (a) the liberation of l-[14C]fucose by a specific α-2-l-fucosidase, (b) its resistance to degradation by alkali and (c) the identification of tritiated glycerol as a product of periodate oxidation after a 3H label had been introduced into the galactosyl moiety. The use of phenyl β-d-galactopyranoside as acceptor substrate thus provides a simple and relatively rapid method for the assay of the blood-group H gene-specified α-2-l-fucosyltransferase.
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| 2. |
- Arvidsson, J, et al.
(författare)
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The first case of the Sanfilippo type C syndrome in Scandinavia
- 1983
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Ingår i: Acta Paediatrica Scandinavica. - : Wiley. - 0001-656X .- 0803-5253 .- 1651-2227. ; 72:2, s. 313-316
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Tidskriftsartikel (refereegranskat)abstract
- A Swedish patient with typical symptoms of the Sanfilippo Syndrome (Mucopolysaccharidosis III) is described. The early onset of the disease, the presence of hepatomegaly, early dementia and the absence of umbilical bernia are consistent with the subgroup Sanfilippo A. Enzyme studies indicate the diagnosis Sanfilippo C, and thus the patient represents a more severe form of this subgroup than any of the four other patients hitherto described in detail.
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| 3. |
- Blomberg, L, et al.
(författare)
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Improved removal of anti-A and anti-B antibodies from plasma using blood-group-active haptens
- 1993
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Ingår i: Vox Sanguinis. - 1423-0410. ; 65:2, s. 126-135
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Tidskriftsartikel (refereegranskat)abstract
- Highly efficient anti-blood group A and B immunoadsorbents for extracorporeal treatment were developed by immobilizing A and B trisaccharide and A tetrasaccharide haptens on Sepharose 4FF and Fractogel TSK using three different methods. The adsorption of the IgG and IgM anti-A antibodies was essentially the same regardless of the A hapten used or the method of oligosaccharide coupling. The adsorption of IgM anti-A was found to be more sensitive than IgG anti-A to changes in column flow rate. The binding of both the IgM and IgG antibodies was slightly lower at 37 degrees C than at 22 degrees C. An anti-A/anti-B adsorbent column potentially suitable for treatment of patients was prepared. A column switching system resulted in a more efficient adsorption of antibodies. Hapten leakage from the column was very low. No nonspecific adsorption of plasma proteins to the column (other than traces of albumin) could be detected.
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| 4. |
- Bodelsson, Mikael, et al.
(författare)
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Differential effect of hypothermia on the vascular tone and reactivity of the human coronary artery and graft vessels
- 1991
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Ingår i: Journal of Cardiovascular Surgery. - 0021-9509. ; 32:3, s. 288-294
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Tidskriftsartikel (refereegranskat)abstract
- Hypothermia may contribute to vascular spasm during bypass surgery. The effect of cooling on the reactivity of the human coronary artery (CA), saphenous vein (SV) and internal mammary artery (IMA) was studied in vitro. In CA and IMA cooling diminished the resting tension and the contraction to potassium, noradrenaline and 5-hydroxytryptamine. In contrast, in SV the contraction to noradrenaline and 5-hydroxytryptamine was augmented by cooling. The effect of cold was reversible. These results demonstrate different effects of hypothermia in CA and the graft vessels. Thus, hypothermia augments the receptor-mediated contraction in SV but depresses it in IMA which thereby resembles CA. The difference is most marked in the contractile response to 5-hydroxytryptamine, which may accumulate during surgery. This may contribute to spasm in the saphenous vein grafts and may be involved in the mechanisms responsible for the inferior patency of SV compared to IMA as a graft vessel.
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| 5. |
- Chester, Alan, et al.
(författare)
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A comparison of the alpha-L-fucosidase activities of human liver and serum
- 1977
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Ingår i: Biochimica et Biophysica Acta. - 0006-3002. ; 485:1, s. 147-155
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Tidskriftsartikel (refereegranskat)abstract
- Human liver alpha-L-fucosidase (alpha-L-fucoside fucohydrolase, EC 3.2.1.51) has been separated into four components by chromatography on Sephadex G-150 or DEAE-cellulose. These components differ in their relative stability to heat and acid treatment, and their response to neuraminidase. The serum enzyme was devoid of high molecular weight activity and probably contained more sialic acid residues than the corresponding enzyme from liver. All the liver components tested were able to liberate fucose from 2'-fucosyllactose but not fucose from other oligosaccharides.
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| 6. |
- Chester, Alan, et al.
(författare)
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A new N-acetyl-beta-D-hexosaminidase disease with late onset of progressive neurological symptoms
- 1979
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Ingår i: Human Heredity. - 1423-0062. ; 29:2, s. 124-128
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Tidskriftsartikel (refereegranskat)abstract
- Clinical data are presented on a 30-year-old male with normal early development (4-5 years) but subsequent progressive impairment of psychomotor functions. He has marked kyphoscoliosis and talipes calcaneo-valgus. The organs appear normal and the patient can walk unaided and feed himself although he does not recognize his parents. He has normal fundi oculi. Biochemical data show an absence of mucopolysacchariduria and very low but detectable levels of N-acetyl-beta-D-hexosaminidase in serum and leucocytes. The clinical symptoms are much milder than would normally be expected from such a profound enzyme deficiency (Sandhoff disease).
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| 7. |
- Chester, Alan, et al.
(författare)
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Alpha-L-fucosyltransferases in human submaxillary gland and stomach tissues associated with the H, Lea and Leb blood-group characters and ABH secretor status
- 1969
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Ingår i: Biochemical and Biophysical Research Communications. - 1090-2104. ; 34:6, s. 835-842
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Tidskriftsartikel (refereegranskat)abstract
- Schemes for the biosynthetic steps in the formation of the blood group active glycoproteins in secretions postulate that α-Image -fucosyltransferases specified by the Image and Image genes control the addition of Image -fucose to different acceptor sites in a precursor substance to give H, Lea and Leb serologically active structures. 1,2. The presence of 2-, 3- and 4-α-Image -fucosyl-transferases in submaxillary glands and stomach mucosal linings from persons who are secretors of ABH, and of 3- and 4-α-Image -fucosyltransferases in tissues from non-secretor persons, is described in this paper.
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| 8. |
- Chester, Alan, et al.
(författare)
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Biosynthesis of the blood-group-B-specific trisaccharide in a rhesus monkey
- 1977
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Ingår i: European Journal of Biochemistry. - 0014-2956. ; 77:1, s. 87-91
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Tidskriftsartikel (refereegranskat)abstract
- A Rhesus monkey, serologically grouped as B, has been shown to excrete low-molecular-weight carbohydrate material in urine closely related to that found in human urine. Galactose feeding resulted in the excretion of a trisaccharide which was shown to be identical to the trisaccharide isolated from the urine of group B humans under the same conditions. Experiments in which [14C]galactose was administered both orally and via an intestinal vein demonstrated that the intestine is the site of biosynthesis.
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| 9. |
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| 10. |
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