Chromatographic separation of putative precursors of cholestanol
This paper describes convenient syntheses for labeled and unlabeled cholest-5-en-3-one, cholest-4-en-3-one, epicholesterol, cholest-4-en-3 beta-ol, and cholest-4-en-3 alpha-ol. The thin-layer chromatography, high-performance liquid chromatography, and gas-liquid chromatography of these compounds and of cholestanol and epicholestanol are also described. The synthesized compounds are potential precursors in the biosynthesis of cholestanol in mammals.
Biosynthesis of 5 -cholestan-3 -ol in cerebrotendinous xanthomatosis.
Cerebrotendinous Xanthomatosis is a rare, inherited disease characterized by an extraordinary accumulation of cholestanol in all tissues, xanthomatous deposits in the brain, lungs, and Achilles tendons, premature atherosclerosis, and low plasma cholesterol concentrations. In two patients with the disease, the biosynthesis of cholestanol was examined by different techniques. After cholesterol-4-(14)C was injected intravenously into one patient, cholestanol and cholesterol isolated from the bile on 3 different days over the ensuing week contained significant radioactivity. The specific radioactivity-time curves for cholesterol-(14)C and cholestanol-(14)C suggested a precursor product relationship and provided additional evidence for the transformation of cholesterol into cholestanol. The second patient received intravenously a mixture of mevalonate-2-(14)C and stereospecifically labeled mevalonate-3R,4R-(3)H. Again cholesterol and cholestanol were isolated from the bile, and the (3)H/(14)C ratio in both sterols was almost the same. This experiment again demonstrated that the biosynthetic path of cholestanol proceeded through cholesterol and not directly from earlier 5alpha-H-saturated precursors. These two independent lines of evidence indicate that the extraordinary deposition of cholestanol in Cerebrotendinous Xanthomatosis arises from cholesterol presumably through the accentuation of the normal biosynthetic pathway.
A Critical Assessment on Functional Attributes and Degradation Mechanism of Membrane Electrode Assembly Components in Direct Methanol Fuel Cells
Direct methanol fuel cells (DMFC) are typically a subset of polymer electrolyte membrane fuel cells (PEMFC) that possess benefits such as fuel flexibility, reduction in plant balance, and benign operation. Due to their benefits, DMFCs could play a substantial role in the future, specifically in replacing Li-ion batteries for portable and military applications. However, the critical concern with DMFCs is the degradation and inadequate reliability that affect the overall value chain and can potentially impede the commercialization of DMFCs. As a consequence, a reliability assessment can provide more insight into a DMFC component’s attributes. The membrane electrode assembly (MEA) is the integral component of the DMFC stack. A comprehensive understanding of its functional attributes and degradation mechanism plays a significant role in its commercialization. The methanol crossover through the membrane, carbon monoxide poisoning, high anode polarization by methanol oxidation, and operating parameters such as temperature, humidity, and others are significant contributions to MEA degradation. In addition, inadequate reliability of the MEA impacts the failure mechanism of DMFC, resulting in poor efficiency. Consequently, this paper provides a comprehensive assessment of several factors leading to the MEA degradation mechanism in order to develop a holistic understanding.