polyol profiles in body fluids have been reported, but the origins of these polyols are unknown. We hypothe-sized that they are derived from sugar phosphate inter-mediates of the pentose phosphate pathway (PPP), and we developed a semiquantitative method for profiling of pentose phosphate pathway intermediates. Methods: Sugar phosphates in blood spots were simul-taneously analyzed by liquid chromatography–tandem mass spectrometry using an ion-pair-loaded C18 HPLC column. The tandem mass spectrometer was operated in the multiple-reaction monitoring mode. Enzymatically prepared D-[13C6]glucose 6-phosphate was used as inter-nal standard. The method was used to study sugar phosphates abnormalities in a patient affected with a deficiency of tran...
Phosphomannomutase (PMM) deficiency causes congenital disorder of glycosylation (CDG)-Ia, a broad sp...
The goal of this research is to understand the role of organic phosphates in the initial step of the...
BACKGROUND: Urinary excretion of the tetrasaccharide 6-alpha-D-glucopyranosyl-maltotriose (Glc(4)) i...
We describe a liquid chromatography tandem mass spectrometry (LC-MS/MS) method to quantify pentose p...
To access publisher's full text version of this article click on the hyperlink belowInborn errors of...
This article describes the first patient with a deficiency of transaldolase (TALDO1 [E.C.2.2.1.2]). ...
This article describes the first patient with a deficiency of transaldolase (TALDO1 [E.C.2.2.1.2]). ...
Abstract Transaldolase (TALDO) deficiency is a rare meta-bolic disease in the pentose phosphate path...
<p>(A) PPP in BSF <i>T</i>. <i>brucei</i>. Black font = detected on LCMS, grey font = inferred inter...
The pentose phosphate pathway (PPP) is a central pathway of the cellular carbohydrate metabolism. Th...
ABSTRACT: We report new substrates for quantitative enzyme activity measurements of human palmitoyl ...
Background: The development of therapies for lysoso-mal storage disorders has created a need for bio...
dehyde-3-phosphate via transaldolase modifies hepatic G6P enrichment from glucose or gluconeogenic t...
BackgroundThe development of therapies for lysosomal storage disorders has created a need for bioche...
Item does not contain fulltextBACKGROUND: Sedoheptulose, arabitol, ribitol, and erythritol have been...
Phosphomannomutase (PMM) deficiency causes congenital disorder of glycosylation (CDG)-Ia, a broad sp...
The goal of this research is to understand the role of organic phosphates in the initial step of the...
BACKGROUND: Urinary excretion of the tetrasaccharide 6-alpha-D-glucopyranosyl-maltotriose (Glc(4)) i...
We describe a liquid chromatography tandem mass spectrometry (LC-MS/MS) method to quantify pentose p...
To access publisher's full text version of this article click on the hyperlink belowInborn errors of...
This article describes the first patient with a deficiency of transaldolase (TALDO1 [E.C.2.2.1.2]). ...
This article describes the first patient with a deficiency of transaldolase (TALDO1 [E.C.2.2.1.2]). ...
Abstract Transaldolase (TALDO) deficiency is a rare meta-bolic disease in the pentose phosphate path...
<p>(A) PPP in BSF <i>T</i>. <i>brucei</i>. Black font = detected on LCMS, grey font = inferred inter...
The pentose phosphate pathway (PPP) is a central pathway of the cellular carbohydrate metabolism. Th...
ABSTRACT: We report new substrates for quantitative enzyme activity measurements of human palmitoyl ...
Background: The development of therapies for lysoso-mal storage disorders has created a need for bio...
dehyde-3-phosphate via transaldolase modifies hepatic G6P enrichment from glucose or gluconeogenic t...
BackgroundThe development of therapies for lysosomal storage disorders has created a need for bioche...
Item does not contain fulltextBACKGROUND: Sedoheptulose, arabitol, ribitol, and erythritol have been...
Phosphomannomutase (PMM) deficiency causes congenital disorder of glycosylation (CDG)-Ia, a broad sp...
The goal of this research is to understand the role of organic phosphates in the initial step of the...
BACKGROUND: Urinary excretion of the tetrasaccharide 6-alpha-D-glucopyranosyl-maltotriose (Glc(4)) i...