Ribitol, a reduced sugar, is a crystalline pentose alcohol formed by the reduction of ribose. Enhancing the flux of D-glucose to the pentose phosphate pathway in Saccharomyces cerevisiae for the production of D-ribose and ribitol[3]. Ribitol, considered to be a metabolic end-product, enhances matriglycan expression in dystrophic muscles in vivo. Ribitol is able to significantly enhance the expression of matriglycan on α-DG in breast cancer cells. The ribitol effect is associated with an increase in levels of CDP-ribitol, the substrate for the ribitol-5-phosphate transferases FKRP and FKTN. Direct use of CDP-ribitol is also effective for matriglycan expression. Ribitol treatment does not alter the expression of FKRP, FKTN as well as LARGEs and ISPD which are critical for the synthesis of matriglycan[4]. Oral administration of ribitol restores therapeutic levels of functional glycosylation of α-DG (F-α-DG) in a FKRP mutant mouse model. Furthermore, the combined approach of ribitol and ISPD acts synergistically, increasing F-α-DG up to 40% of normal levels in cardiac muscle and more than 20% in limb and diaphragm[5].
搜索
