Determination of a mannose-containing trisaccharide in the urine of patients and heterozygotes of mannosidosis

Lundblad, Arne; Masson, P; Nordén, Nils E.; Svensson, Sigfrid; Öckerman, P.A.

doi:10.1002/bms.1200020602

Cited by 14 publications

(7 citation statements)

References 11 publications

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“…Established methods for the analysis of these compounds are time-consuming and do not necessarily allow good separation and recovery of individual components. Gas-liquid chromatography/mass spectrometry has proved to be an ideal method for rapid and accurate determinations of different oligosaccharides in complex biological materials [16,19] and was therefore used in the present study. Ultrafiltered deionized urine was reduced and permethylated directly in most cases.…”

Section: Discussionmentioning

confidence: 99%

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Urinary Excretion of Oligosaccharides Induced by Galactose Given Orally or Intravenously

Chester¹,

Hallgren²,

Lundblad³

et al. 1979

Eur J Biochem

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The effect of oral administration of galactose, lactose, and sucrose and intravenous injection of galactose on the urinary excretion of blood-group-active oligosaccharides has been studied. Galactose given either as the free sugar, a glycoside (lactose) or a constituent of normal diet was an absolute requirement for the formation and excretion of A-trisaccharide, B-trisaccharide and 2'-fucosylgalactose in blood group A, B and O(H) secretors, respectively. Great individual variation was seen in the amounts of galactose-dependent oligosaccharides excreted. Injection of galactose resulted in excretion of 3 -59 of the amount of oligosaccharide formed after oral administration to the same individual. The mean ratio A-trisaccharide/B-trisaccharide was 2.7 in four blood-group-AIB secretors and 0.22 in three A2B secretors and can thus serve as a parameter for chemical differentiation between the two blood groups. The excretion of larger blood-group-active oligosaccharides, including the A-pentasaccharide, the B-pentasaccharide and lactodifucotetraose, that are normal components in urine from, respectively, starved A, B, and H secretors, was about the same after oral administration of galactose or lactose. The B-trisaccharide wdS the only oligosaccharide detected in plasma after oral galactose administration to a blood-group-B secretor individual. The concentration was 0.38 mg/l of plasma.Blood-group-active tetrasaccharides and pentasaccharides have been isolated from the urine of starved blood-group A, B or H secretors [l -41. Normal food intake or lactose diet induced an excretion of additional blood-group-active disaccharides or trisaccharides in these urines [5]. The oligosaccharides formed by A, B and H secretors are shown in Fig.

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Section: Discussionmentioning

confidence: 99%

“…Methylation of reduced (NaB2H4) oligosaccharides was carried out as described elsewhere [15,16]. Permethylated oligosaccharides were separated as their alditol derivatives on a Perkin-Elmer 3920 gas chromatograph equipped with a glass capillary column (25 m x 0.25 mm), wall-coated with SE-30 (LKB, Stockholm, Sweden).…”

Section: Methodsmentioning

confidence: 99%

Urinary Excretion of Oligosaccharides Induced by Galactose Given Orally or Intravenously

Chester¹,

Hallgren²,

Lundblad³

et al. 1979

Eur J Biochem

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“…The reduced oligosaccharide was then hydrolysed with 0.25 M aqueous sulphuric acid (5 ml) at 100 C for 1 h, cooled and neutralised with barium carbonate. The partial hydrolysate was finally reduced with sodium borodeuteride, permethylated [32] and analysed by gas-liquid chromatography -mass spectrometry.…”

Section: Aizaly T Im1 Me Thomentioning

confidence: 99%

Urinary Abnormalities in Fucosidosis. Characterization of a Disaccharide and Two Glycoasparagines

Lundblad¹,

Lundsten²,

Nordén³

et al. 1978

Eur J Biochem

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The urinary excretion of fucose‐containing material was found to be highly increased in a patient with fucosidosis type 2. Three structurally related compounds, a disaccharide and two glycoasparagines, were isolated from the urine. The isolation procedure included ultrafiltration. gel chromatography on Sephadex G‐25, preparative zone electrophoresis and paper chromatography. From structural studies including optical rotation, sugar analysis, methylation analysis, ninhydrin degradation, reduction with lithium aluminium hydride and partial hydrolysis, the following structures were deduced: where Fucp is fucopyranose, Manp is mannopyranose, Galcp is galactopyranose, GlcNAcp is 2‐acetamido‐2‐deoxyglucopyranose and Asn is asparagine. The yields of these compounds were 1.7, 40, and 6 mg/l, respectively. The origin of the disaccharide and the two glycoasparagines is probably the core region of glycoprotein carbohydrate chains.

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“…Gas-liquid chromatography-mass spectrometry (GC-MS) has been used to detect specific compounds known to be excreted in some glycogen storage diseases and muscular dystrophies (Lennartson et at., 1976) and in mannosidosis (Lundblad et al, 1975). This method has now been extended for use as a general screening method for the detection of low molecular weight carbohydrate-containing compounds.…”

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confidence: 96%

“…The amounts of these compounds excreted are usually quite low ( < 20 rag/24 h). In certain diseases additional compounds are excreted in much larger amounts, for example in mannosidosis a mannose-containing trisaccharide and tetrasaccharide are excreted in quantities up to 470 and 60 mg/24 h respectively (Lundblad et al, 1975; Norddn et al, 1974) and a glucose-containing tetrasaccharide is excreted in amounts approaching 90 mg/24 h in certain glycogen storage diseases (Lennartson et al, 1976).The overall picture therefore is of a complex mixture of low molecular weight carbohydrate-containing material which can show considerable non-pathological variation, but in at least some pathological states this pattern is overshadowed by great excesses of specific compounds or series of related compounds.The basic procedure developed to analyse urinary oligosaccharides involves desalting 20 ml of ultrafiltered urine through Dowex 50 (H +) and AG-3 (OH-) resins, fbltowed by reduction of the oligosaccharides with sodium borodeuteride and methylation (Lundblad et al, 1975). The methylated and reduced oligosaccharides are then separated by gas-liquid chromatography (GLC ; Perkin-Elmer 3920 : capillary column, 25 m x 0.25 mm, wall-coated with SE-30; temperature programme 230 °C/30 min, 230 °C 330 °C at 4 °C/rain, 330 °C/30 min) and identified by mass spectrometry (Varian MAT 311A, 70 eV, 1 mA, ion source 120°C) linked to a Spectrosystem 100 computer.…”

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confidence: 99%

Isolation and characterization of oligosaccharides from urine of patients with abnormal glycoconjugate metabolism

Chester

Lundblad

Svensson

et al. 1978

J of Inher Metab Disea

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Diseases involving abnormal glycoconjugation are frequently diagnosed by enzymic methods of varying complexity (Table 1) and/or by analysis of abnormally excreted carbohydrate-containing material. Gas-liquid chromatography-mass spectrometry (GC-MS) has been used to detect specific compounds known to be excreted in some glycogen storage diseases and muscular dystrophies (Lennartson et at., 1976) and in mannosidosis (Lundblad et al., 1975). This method has now been extended for use as a general screening method for the detection of low molecular weight carbohydrate-containing compounds.A number of oligosaccharides are present in normal human urine and include lactose, maltose, isomaltose, fucosyl-a-( 1-2)-glucose, xylosyl-a-( 1-3)-glucose, fucosyl-~-(1-O)-inositol, a glucose-containing tetrasaccharide and a series of disaccharides to pentasaccharides, the presence of which depends on ABO blood group, secretor status and diet (Lundblad et al., 1973). The amounts of these compounds excreted are usually quite low ( < 20 rag/24 h). In certain diseases additional compounds are excreted in much larger amounts, for example in mannosidosis a mannose-containing trisaccharide and tetrasaccharide are excreted in quantities up to 470 and 60 mg/24 h respectively (Lundblad et al., 1975; Norddn et al., 1974) and a glucose-containing tetrasaccharide is excreted in amounts approaching 90 mg/24 h in certain glycogen storage diseases (Lennartson et al., 1976).The overall picture therefore is of a complex mixture of low molecular weight carbohydrate-containing material which can show considerable non-pathological variation, but in at least some pathological states this pattern is overshadowed by great excesses of specific compounds or series of related compounds.The basic procedure developed to analyse urinary oligosaccharides involves desalting 20 ml of ultrafiltered urine through Dowex 50 (H +) and AG-3 (OH-) resins, fbltowed by reduction of the oligosaccharides with sodium borodeuteride and methylation (Lundblad et al., 1975). The methylated and reduced oligosaccharides are then separated by gas-liquid chromatography (GLC ; Perkin-Elmer 3920 : capillary column, 25 m x 0.25 mm, wall-coated with SE-30; temperature programme 230 °C/30 min, 230 °C 330 °C at 4 °C/rain, 330 °C/30 min) and identified by mass spectrometry (Varian MAT 311A, 70 eV, 1 mA, ion source 120°C) linked to a Spectrosystem 100 computer. The resulting chromatogram contains a large number of peaks grouped in the monosaccharide and disaccharide regions, but the trisaccharide to pentasaccharide region is usually dominated by a few compounds as shown for a normal blood group B secretor individual in Figure 1. The major component is the blood group B trisaccharide (Lundblad et aI., 1973). Also shown is the glucosecontaining tetrasae.charide (Glq). By comparison, the same region of the chromatogram is shown for urine from a mannosidosis patient in Figure 2. The only significant peaks in this case are two of the mannosecontaining oligosaccharides which cannot be degraded by...

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Determination of a mannose-containing trisaccharide in the urine of patients and heterozygotes of mannosidosis

Cited by 14 publications

References 11 publications

Urinary Excretion of Oligosaccharides Induced by Galactose Given Orally or Intravenously

Urinary Excretion of Oligosaccharides Induced by Galactose Given Orally or Intravenously

Urinary Abnormalities in Fucosidosis. Characterization of a Disaccharide and Two Glycoasparagines

Isolation and characterization of oligosaccharides from urine of patients with abnormal glycoconjugate metabolism

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