bims-meglyc 2026-10-04 papers

Issue of 2026–10–04
two papers selected by
Silvia Radenkovic, UMC Utrecht

J Inherit Metab Dis. 2026 Nov;49(6): e70256

Congenital Disorders of Glycosylation (CDG): State of the Art in 2026.

Congenital disorders of glycosylation (CDG) are a rapidly expanding group of inherited metabolic diseases affecting glycoconjugate glycan biosynthesis and attachment. This review provides a structured overview of major advances in human CDG reported from 2023 to 2026, including newly recognized CDG, emerging biochemical and pathophysiological insights, advances in diagnostic strategies and biomarkers, and progress in treatment. Recent developments include the recognition of RPN1-CDG, UGGT1-CDG, and DHRSX-CDG, refinement of phenotypes in established disorders, including PMM2-CDG, ALG13-CDG, ALG8-CDG, GMPPA-CDG, TRAPPC11-CDG, and PGAP3-CDG, and increasing use of glycomics, glycoproteomics, cellular models, and multi-omics approaches. Therapeutic progress remains uneven: no FDA/EMA-approved disease-modifying therapy is currently available for any CDG, and most interventions remain off-label, experimental, or supported by limited observational evidence. Despite substantial progress, most CDG still lack disease-modifying treatment, validated biomarkers, and prospective natural history data.

Keywords: congenital disorders of glycosylation; glycomics; inherited metabolic diseases; molecular diagnosis; targeted therapy

DOI: https://doi.org/10.1002/jimd.70256

Brain Res Bull. 2026 Sep 26. pii: S0361-9230(26)00431-4. [Epub ahead of print]245 112144

Glycosylation in neurological disorders: Mechanisms, crosstalk, and therapeutic implications.

Wenbo Wu, Wanyu Wang, Lintao Xu, Ying Yao, Xinyue Zhao, Jinjie Zhong, Jue Hu, Linlin Wang, Yongjian Zhu, Jingyu Wang.

Neurological disorders pose a major global health challenge for complicated pathogenesis and a lack of effective curative treatments. As a post-translational modification, glycosylation has emerged as a multifunctional regulator in the nervous system, influencing key processes including receptor activation, cell adhesion, and immune surveillance. Its dysfunction is increasingly recognized as a shared mechanism across various neurological disorders. This review synthesizes the molecular mechanisms of glycosylation within a novel framework of four neurological disorder categories: neurodevelopmental disorders, proteostasis disorders, neuroimmunological disorders, and acute nerve injuries. Furthermore, we advance the concept of glycosylation as an integrator of mechanistic crosstalk and demonstrate its pivotal role in bridging and amplifying intertwined pathological cascades. This integrative perspective aims to reframe the understanding of disease pathogenesis and inspire innovative diagnostic paradigms and therapeutic strategies.

Keywords: Acute Nerve Injuries; Glycosylation; Mechanistic Crosstalk; Neurodevelopmental Disorders; Neuroimmunological Disorders; Proteostasis Disorders

DOI: https://doi.org/10.1016/j.brainresbull.2026.112144