Different approaches have been utilized or proposed for the treatment of lysosomal storage disorders (LSDs) including enzyme replacement and hematopoietic stem cell transplant therapies, both aiming to compensate for the enzymatic loss of the underlying mutated lysosomal enzymes. However, these approaches have their own limitations and therefore the vast majority of LSDs are either still untreatable or their treatments are inadequate. Missense mutations affecting enzyme stability, folding and cellular trafficking are common in LSDs resulting often in low protein half-life, premature degradation, aggregation and retention of the mutant proteins in the endoplasmic reticulum. Small molecular weight compounds such as pharmaceutical chaperones (PCs) and proteostasis regulators have been in recent years to be promising approaches for overcoming some of these protein processing defects. These compounds are thought to enhance lysosomal enzyme activity by specific binding to the mutated enzyme or by manipulating components of the proteostasis pathways promoting protein stability, folding and trafficking and thus enhancing and restoring some of the enzymatic activity of the mutated protein in lysosomes. Multiple compounds have already been approved for clinical use to treat multiple LSDs like migalastat in the treatment of Fabry disease and others are currently under research or in clinical trials such as Ambroxol hydrochloride and Pyrimethamine. In this review, we are presenting a general overview of LSDs, their molecular and cellular bases, and focusing on recent advances on targeting and manipulation proteostasis, including the use of PCs and proteostasis regulators, as therapeutic targets for some LSDs. In addition, we present the successes, limitations and future perspectives in this field.

The underutilized Kenyan variety of Dolichos lablab bean seeds serves as a good source of nutrients. This study was undertaken for the first time to address the limited knowledge regarding the bioactive, biochemical, physicochemical, and functional properties of Dolichos lablab beans. Proximate analysis, mineral, total and free amino acid profiles, total protein, total dietary and profile of fiber, carotenoids and fat soluble vitamins, total phenolics, and total flavonoids were evaluated. The results clearly show that Dolichos lablab beans contain many health-promoting components, such as fiber, proteins, minerals, and numerous phytochemicals endowed with useful biological activities, that allow it to contribute in a relevant way to the daily intake of these nutrients.

The underutilized Kenyan variety of Dolichos lablab bean seeds serve as a good source of natural antioxidants, which can probably be effective in reducing the risk of occurrence of several diseases. This study was undertaken for the first time to address the limited knowledge regarding the antioxidant activities of lablab beans. Moreover, their DNA damage inhibitory activity, bovine serum albumin (BSA) damage inhibitory activity, and the inhibition of acetylcholinesterase and porcine α-amylase were also investigated. The antioxidant capacity of Dolichos lablab bean seeds extracted with methanol, water or methanol/water combination was evaluated by the ferric-reducing antioxidant power (FRAP) assay, free radical-scavenging activity, 1,1-diphenyl-2-picrylhydrazyl (DPPH), nitric oxide (NO) radical-scavenging assay, and 2,20-azinobis(3-ethylbenzothiazoline-6-sulfonic acid) (ABTS). Results reported in the present study indicate that water, methanol and water/methanol extracts of lablab bean flour exhibited good antioxidant activity by effectively scavenging various free radicals, such as DPPH, NO, and ABTS radicals. The extracts also exhibited protective effects against DNA and BSA damage and inhibitory effects on porcine α-amylase. Findings of this study suggest that extracts from the lablab bean flour would have potential application in food supplements, and pharmaceutical and cosmetic industries.

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This underutilized variety of Dolichos lablab bean seed serves as a good source of natural antioxidants, which may be effective in reducing the risk of occurrence of certain diseases. This study was undertaken for the first time to address the limited knowledge regarding the, antioxidant activities, DNA damage inhibitory activity, bovine serum albumin (BSA) damage inhibitory activity, anti‐acetylcholinesterase, and anti‐porcine α–amylase in the lablab bean seed. The antioxidant capacity of lablab bean seeds were evaluated for their potential antioxidant activities using the ferric‐reducing/antioxidant power assay (FRAP), free radical‐scavenging activity (DPPH), nitric oxide (NO) radical‐scavenging assay, and 2,20‐azinobis(3‐ethylbenzothiazoline‐6‐sulfonic acid (ABTS). The lablab bean extract exhibited good antioxidant activity by effectively scavenging FRAP, DPPH, NO, and ABTS radicals. Additionally, excellent protective effects against BSA damage, as well as natural α‐amylase inhibitors were also shown. The bean extract had protective effects against DNA damage. The lablab bean may have a future role as an ingredient in the development of functional foods