BIOMAT Database Logo BIOMAT Database Logo

The structures of six urinary oligosaccharides that are characteristic for a patient with Morquio syndrome type B. 1982

J C Michalski, and G Strecker, and H van Halbeek, and L Dorland, and J F Vliegenthart

Morquio syndrome type B is an inherited, lysosomal storage disease characterised by a marked deficiency in acid beta-D-galactosidase, while the 2-acetamido-2-deoxy-beta-D-galactose 6-sulphate sulphatase activity is normal. Urinary oligosaccharides were studied in order to evaluate the effect of the diminished beta-D-galactosidase activity on the catabolism of glycoconjugates and to compare their structure with those excreted by patients with GM1-gangliosidosis. The following oligosaccharides were isolated: beta-D-Galp-(1 leads to 4)-beta-D-GlcpNAc-(1 leads to 2)-alpha-D-Manp-(1 leads to 6)-beta-D-Manp-(1 leads to 4)-D-GlcpNac(1), beta-D-Galp-(1 leads to 4)-beta-D-GlcpNAc-(1 leads to 2)-alpha-D-Manp-(1 leads to 6)-[alpha-D-Manp-(1 leads to 3)]-beta-D-Manp-(1 leads to 4)-D-GlcpNAc (2a), beta-D-Galp-(1 leads to 4)-beta-D-GlcpNAc-(1 leads to 2)-alpha-D-Manp-(1 leads to 3)-[alpha-D-Manp-(1 leads to 6)]-beta-D-Manp-(1 leads to 4)-D-GlcpNAc (2b), beta-D-Galp-(1 leads to 4)-beta-D-GlcpNAc-(1 leads to 2)-alpha-D-Manp-(1 leads to 3)-[beta-D-Galp-(1 leads to 4)-beta-D-GlcpNAc-(1 leads to 2)-alpha-D-Manp-(1 leads to 6)]-beta-D-Manp-(1 leads to 4)-D-GlcpNAc (3), beta-D-Galp-(1 leads to 4)-beta-D-Glcp-NAc-(1 leads to 2)-alpha-D-Manp-(1 leads to 3)-(beta-D-Galp-(1 leads to 4)-beta-D-GlcpNAc-(1 leads to 2)-[beta-D-Galp-(1 leads to 4)-beta-D-GlcpNAc-(1 leads to 6)]-alpha-D-Manp-(1 leads to 6))-beta-D-Manp-(1 leads to 4)-D-GlcpNAc (4), beta-D-Galp-(1 leads to 4)-beta-D-GlcpNAc-(1 leads to 2)-alpha-D-Manp-(1 leads to 3)-[beta-D-GlcpNAc-(1 leads to 4)]-[beta-D-Glap-(1 leads to 4)-beta-D-GlcpNAc-(1 leads to 2)-alpha-D-Manp-(1 leads to 6)]-beta-D-Manp-(1 leads to 4)-D-Glcp-NAc (5). Significant differences between Morquio syndrome type B and GM1- gangliosidosis have been observed, with regard to the excretion rate and the specific structures of urinary oligosaccharides. Compounds 2a, 2b, and 5 are novel members of the series of oligosaccharides isolated from the urine of patients with inherited lysosomal storage diseases.

UI MeSH Term Description Entries
D009085 Mucopolysaccharidosis IV Genetic disorder of mucopolysaccharide metabolism characterized by skeletal abnormalities, joint instability, development of cervical myelopathy, and excessive urinary keratan sulfate. There are two biochemically distinct forms, each due to a deficiency of a different enzyme. Eccentro-Osteochondrodysplasia,Morquio's Disease,Eccentroosteochondrodysplasia,GALNS Deficiency,Galactosamine-6-Sulfatase Deficiency,MPS IV A,MPS IV B,MPS IVA,MPS IVB,Morquio A Disease,Morquio B Syndrome,Morquio Disease,Morquio Syndrome,Morquio Syndrome A,Morquio Syndrome B,Morquio Syndrome, Type B,Morquio's Disease Type B,Morquio's Syndrome,Morquio-B Disease,Mucopolysaccharidosis 4,Mucopolysaccharidosis 4B,Mucopolysaccharidosis Type IV,Mucopolysaccharidosis Type IV A,Mucopolysaccharidosis Type IV B,Mucopolysaccharidosis Type IVA,Mucopolysaccharidosis Type IVB,Deficiencies, GALNS,Deficiencies, Galactosamine-6-Sulfatase,Deficiency, GALNS,Deficiency, Galactosamine-6-Sulfatase,Disease, Morquio,Disease, Morquio A,Disease, Morquio's,Disease, Morquio-B,Eccentro Osteochondrodysplasia,Eccentro-Osteochondrodysplasias,Eccentroosteochondrodysplasias,GALNS Deficiencies,Galactosamine 6 Sulfatase Deficiency,Galactosamine-6-Sulfatase Deficiencies,IV, Mucopolysaccharidosis Type,IVs, Mucopolysaccharidosis Type,Morquio B Disease,Morquio Syndromes,Morquios Disease,Morquios Syndrome,Mucopolysaccharidosis 4Bs,Mucopolysaccharidosis Type IVBs,Mucopolysaccharidosis Type IVs,Syndrome A, Morquio,Syndrome, Morquio,Syndrome, Morquio B,Syndrome, Morquio's,Syndromes, Morquio,Type IV, Mucopolysaccharidosis,Type IVs, Mucopolysaccharidosis
D009682 Magnetic Resonance Spectroscopy Spectroscopic method of measuring the magnetic moment of elementary particles such as atomic nuclei, protons or electrons. It is employed in clinical applications such as NMR Tomography (MAGNETIC RESONANCE IMAGING). In Vivo NMR Spectroscopy,MR Spectroscopy,Magnetic Resonance,NMR Spectroscopy,NMR Spectroscopy, In Vivo,Nuclear Magnetic Resonance,Spectroscopy, Magnetic Resonance,Spectroscopy, NMR,Spectroscopy, Nuclear Magnetic Resonance,Magnetic Resonance Spectroscopies,Magnetic Resonance, Nuclear,NMR Spectroscopies,Resonance Spectroscopy, Magnetic,Resonance, Magnetic,Resonance, Nuclear Magnetic,Spectroscopies, NMR,Spectroscopy, MR
D009844 Oligosaccharides Carbohydrates consisting of between two (DISACCHARIDES) and ten MONOSACCHARIDES connected by either an alpha- or beta-glycosidic link. They are found throughout nature in both the free and bound form. Oligosaccharide
D002236 Carbohydrate Conformation The characteristic 3-dimensional shape of a carbohydrate. Carbohydrate Linkage,Carbohydrate Conformations,Carbohydrate Linkages,Conformation, Carbohydrate,Conformations, Carbohydrate,Linkage, Carbohydrate,Linkages, Carbohydrate
D002240 Carbohydrate Sequence The sequence of carbohydrates within POLYSACCHARIDES; GLYCOPROTEINS; and GLYCOLIPIDS. Carbohydrate Sequences,Sequence, Carbohydrate,Sequences, Carbohydrate
D002849 Chromatography, Gas Fractionation of a vaporized sample as a consequence of partition between a mobile gaseous phase and a stationary phase held in a column. Two types are gas-solid chromatography, where the fixed phase is a solid, and gas-liquid, in which the stationary phase is a nonvolatile liquid supported on an inert solid matrix. Chromatography, Gas-Liquid,Gas Chromatography,Chromatographies, Gas,Chromatographies, Gas-Liquid,Chromatography, Gas Liquid,Gas Chromatographies,Gas-Liquid Chromatographies,Gas-Liquid Chromatography
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

Related Publications

J C Michalski, and G Strecker, and H van Halbeek, and L Dorland, and J F Vliegenthart
Anaesthesia for urinary diversion with ileal conduit in a patient with Morquio-Brailsford syndrome.
December 1993, Anaesthesia and intensive care,
J C Michalski, and G Strecker, and H van Halbeek, and L Dorland, and J F Vliegenthart
Two urinary oligosaccharides characteristic of A1 and B secretors: isolation and partial characterization.
October 1967, Biochimica et biophysica acta,
J C Michalski, and G Strecker, and H van Halbeek, and L Dorland, and J F Vliegenthart
Widespread Vasculopathy in a Patient with Morquio A Syndrome.
December 2017, Texas Heart Institute journal,
J C Michalski, and G Strecker, and H van Halbeek, and L Dorland, and J F Vliegenthart
Total knee replacements in a patient with the Morquio syndrome.
December 2008, The Journal of bone and joint surgery. British volume,
J C Michalski, and G Strecker, and H van Halbeek, and L Dorland, and J F Vliegenthart
Structure of two urinary oligosaccharides characteristic of blood group O(H)- and B-secretors.
March 1970, Biochimica et biophysica acta,
J C Michalski, and G Strecker, and H van Halbeek, and L Dorland, and J F Vliegenthart
Aortic valve replacement in a patient with morquio syndrome.
January 2008, The heart surgery forum,
J C Michalski, and G Strecker, and H van Halbeek, and L Dorland, and J F Vliegenthart
Airway management in a patient with Morquio-Brailsford syndrome.
March 2013, European journal of anaesthesiology,
J C Michalski, and G Strecker, and H van Halbeek, and L Dorland, and J F Vliegenthart
Anesthetic management of an adult patient with Morquio A syndrome.
September 2019, Journal of clinical anesthesia,
J C Michalski, and G Strecker, and H van Halbeek, and L Dorland, and J F Vliegenthart
A second reported malignancy in a patient with Morquio syndrome.
January 2017, Autopsy & case reports,
J C Michalski, and G Strecker, and H van Halbeek, and L Dorland, and J F Vliegenthart
Severe rejection of deep anterior lamellar keratoplasty in a patient with mucopolysaccharidosis type IV (Morquio syndrome).
June 2021, Journal francais d'ophtalmologie,
Copied contents to your clipboard!