Coenzyme Q₁₀ and Pyridoxal Phosphate Deficiency Is a Common Feature in Mucopolysaccharidosis Type III.

ABSTRACT: Mucopolysaccharidoses (MPS) are a group of lysosomal storage disorders caused by deficiencies of lysosomal enzymes catalyzing degradation of glycosaminoglycans (GAGs). Previously, we reported a secondary plasma coenzyme Q₁₀ (CoQ) deficiency in MPS patients. For this study, nine MPS patients were recruited in the Hospital Sant Joan de Déu (HSJD, Barcelona) and two patients in the Neurometabolic Unit, National Hospital (NMU, London), to explore the nutritional status of MPS type III patients by analyzing several vitamins and micronutrients in blood and in cerebrospinal fluid. Plasma CoQ and plasma and cerebrospinal fluid pyridoxal phosphate (PLP) content were analyzed by high-pressure liquid chromatography (HPLC) with electrochemical and fluorescence detection, respectively. We fo

SUBMITTER: Yubero D 

PROVIDER: S-EPMC5059182 | biostudies-literature | 2016

REPOSITORIES: biostudies-literature