Glycogen metabolism and structure: A review

GKS Neoh, X Tan, S Chen, E Roura, X Dong… - Carbohydrate …, 2024 - Elsevier
Glycogen is a glucose polymer that plays a crucial role in glucose homeostasis by
functioning as a short-term energy storage reservoir in animals and bacteria. Abnormalities …

Lafora progressive myoclonus epilepsy: Disease mechanism and therapeutic attempts

R Parihar, S Ganesh - Journal of Biosciences, 2024 - Springer
Lafora disease (LD) is a life-threatening autosomal recessive and progressive
neurodegenerative disorder that primarily affects adolescents, resulting in mortality within a …

Myofiber-type-dependent 'boulder'or 'multitudinous pebble'formations across distinct amylopectinoses

S Mitra, B Chen, JM Shelton, S Nitschke, J Wu… - Acta …, 2024 - Springer
At least five enzymes including three E3 ubiquitin ligases are dedicated to glycogen's
spherical structure. Absence of any reverts glycogen to a structure resembling amylopectin …

Implications of a De Novo Variant in the SOX12 Gene in a Patient with Generalized Epilepsy, Intellectual Disability, and Childhood Emotional Behavioral Disorders

S Treccarichi, F Calì, M Vinci, A Ragalmuto… - Current Issues in …, 2024 - mdpi.com
SRY-box transcription factor (SOX) genes, a recently discovered gene family, play crucial
roles in the regulation of neuronal stem cell proliferation and glial differentiation during …

1H and 31P magnetic resonance spectroscopy reveals potential pathogenic and biomarker metabolite alterations in Lafora disease

KL Chan, A Panatpur, S Messahel… - Brain …, 2024 - academic.oup.com
Lafora disease is a fatal teenage-onset progressive myoclonus epilepsy and
neurodegenerative disease associated with polyglucosan bodies. Polyglucosans are long …

Impaired malin expression and interaction with partner proteins in Lafora disease

AV Skurat, DM Segvich, CJ Contreras, YC Hu… - Journal of Biological …, 2024 - jbc.org
Lafora disease (LD) is an autosomal recessive myoclonus epilepsy with onset in the
teenage years leading to death within a decade of onset. LD is characterized by the …

Prognostic value of pathogenic variants in Lafora Disease: systematic review and meta-analysis of patient-level data

F Pondrelli, R Minardi, L Muccioli, C Zenesini… - Orphanet Journal of …, 2023 - Springer
Background Lafora disease (LD) is a fatal form of progressive myoclonic epilepsy caused by
biallelic pathogenic variants in EPM2A or NHLRC1. With a few exceptions, the influence of …

[HTML][HTML] Epm2aR240X knock-in mice present earlier cognitive decline and more epileptic activity than Epm2a−/− mice

DF Burgos, M Sciaccaluga, CA Worby… - Neurobiology of …, 2023 - Elsevier
Lafora disease is a rare recessive form of progressive myoclonic epilepsy, usually
diagnosed during adolescence. Patients present with myoclonus, neurological deterioration …

Supporting the translation of multiscale research in rare disease

KM Hooper, J Hmeljak - Disease Models & Mechanisms, 2023 - journals.biologists.com
Since its launch in 2008, Disease Models & Mechanisms (DMM) has been a welcoming
home for research in rare and ultra-rare disease. Owing to its position at the intersection …

Clinicopathologic Dissociation: Robust Lafora Body Accumulation in Malin KO Mice Without Observable Changes in Home‐Cage Behavior

V Krishnan, J Wu, AG Mazumder… - Journal of …, 2024 - Wiley Online Library
Lafora disease (LD) is a syndrome of progressive myoclonic epilepsy and cumulative
neurocognitive deterioration caused by recessively inherited genetic lesions of EPM2A …