The objectives of the study are to collect natural history and treatment-related data of patients diagnosed with Phosphoglucomutase 1-CDG, MPI-CDG or SLC35C1-CDG who are either treated with or without D-galactose, D-mannose and L-fucose
, respectively, as well as patients with other CDGs who are treated with one of the sugars.
In addition, the chronic inhibition of myo-inositol transport with L-fucose
induces a neuropathy that resembles diabetic neuropathy resulting in decreased nerve conduction velocity, axonal atrophy, paranodal swelling, and demyelination.
Fucoidans are complex sulfated polysaccharides consisting mainly of sulfated L-fucose
and variable proportions of galactose, mannose, xylose, glucose, rhamnose and uronic acids (Usov & Bilan, 2009).
Haplotypes were tested by API 50CHB strips (BioMerieux, Inc., France) for utilization of the following substrates: glycerol, erythritol, D-arabinose, L-arabinose, ribose, D-xylose, L-xylose, Adonitol, B-methyl xyloside, galactose, glucose, fructose, mannose, L-sorbose, rhamnose, dulcitol, inositol, sorbitol, mannitol, L-methyl-D-mannoside, D-methyl-D-glucoside, N-acetylglucosamine, amygdalin, arbutin, aesculin, salicin, cellobiose, maltose, lactose, sucrose, Trehalose, gentiobiose, melibiose, raffinose, melezitose, starch, glycogen, inulin, D-turanose, D-tagatose, D-fucose, L-fucose
, D-lyxose, D-arabitol, L-arabitol, xylitol, gluconate, and 2,5-ketogluconate.
Daniel, "Differentiation of the fucoidan sulfated L-fucose
isomers constituents by CE-ESIMS and molecular modeling," Carbohydrate Research, vol.
Analysis of sugar composition was performed by GC-MS; it indicated that they were composed of L-fucose
, D-mannose, D-glucose, and D-galactose in molar ratios of 1.4: 1: 1.3: 1.7.
glutinins produce a heteropolysaccharide composed of L-fucose
and Dgalactosein an approximate molar ratio of 1:1.
Fucoidan, which is extracted from brown seaweed, is a complex sulphated polysaccharide that is mostly composed of L-fucose
and sulphated ester groups.
The strain reduced nitrate to nitrite, produced N-acetyl-[beta]-glucosamidase, phosphatase, [alpha]- and [beta]-galactosidases, [alpha]- and [alpha]-glucosidases, and assimilated L-arabinose, L-fucose
, D-glucose, maltose, D-mannose, D-melibiose, L-rhamnose, sucrose, and salicin with Api 20NE, Api ID32GN (bioMerieux), and ID-GNB biochemical galleries.
The specificity of lectin binding was tested by addition of free L-fucose
to the AAL solution before addition to the microtiter plate.
Other sugars that are associated with terminal differentiation and apoptosis include those that specify histo-blood group H (L-fucose