RESUMO
This review provides a perspective on the industrial application potential of sugar beet pulp (SBP) derived monosaccharides. The broad application of these monosaccharides could contribute to bio-based alternatives and sustainable practices, essential for the transition towards a more circular economy. This review focuses on the utilization and application of two SBP monosaccharides, d-galacturonic acid (d-GalA) and l-arabinose (l-Ara), derived from pectin and hemicellulose. These polysaccharides are major components of sugar beet pulp, an important side stream of sucrose production. d-GalA and l-Ara are therefore abundant in biomass and offer unique molecular structures amenable to selective chemical or enzymatic modifications. We review their application in various industrial applications such as the development and production of bioactive compounds, home and personal care products, and other industries.
RESUMO
Hyperprolinemia type II (HPII) is an inborn error of metabolism due to genetic variants in ALDH4A1, leading to a deficiency in Δ-1-pyrroline-5-carboxylate (P5C) dehydrogenase. This leads to an accumulation of toxic levels of P5C, an intermediate in proline catabolism. The accumulating P5C spontaneously reacts with, and inactivates, pyridoxal 5'-phosphate, a crucial cofactor for many enzymatic processes, which is thought to be the pathophysiological mechanism for HPII. Here, we describe the use of a combination of LC-QTOF untargeted metabolomics, NMR spectroscopy and infrared ion spectroscopy (IRIS) to identify and characterize biomarkers for HPII that result of the spontaneous reaction of P5C with malonic acid and acetoacetic acid. We show that these biomarkers can differentiate between HPI, caused by a deficiency of proline oxidase activity, and HPII. The elucidation of their molecular structures yields insights into the disease pathophysiology of HPII.