Introduction: LaFora Disease (LD) is a rare form of progressive infantile epilepsy where rapid neurological damage that occurs as the disease advances, which causes the patient to a vegetative state and then die, usually within the first decade of the onset of illness. Based on the capacity of the endogenous cannabinoid system (ECS) to modulate several cellular processes commonly altered in many neurodegenerative processes, as well as the antiepileptic properties of certain natural cannabinoids, the aim of this study was to evaluate the role of ECS in LD developments.

Materials and Methods: We tested whether natural cannabis extract highly enriched in cannabidiol (CBD) may be effective in controlling the pathological phenotype master knockout (KO) mice as an animal model LD. Results: Our results show for the first time that changes in the ECS occurred during the evolution of LD, especially at the level of CB1, CB2, and G protein-coupled receptor-coupled 55 (GPR55) receptor expression, and that the extract CBD-enriched (CBDext) is able to reduce interference cognitive demonstrated by master KO mice.

However, contrary to what has previously been reported for other types of refractory epilepsy in childhood, CBD-enriched extract did not reduce the severity of epileptic seizures induced in animal models of LD. Conclusion: In summary, this study revealed that the ECS may play a role in the LD and that the CBD-enriched extract partially alleviate dementia like phenotype, but did not increase susceptibility to epileptic seizures, exhibited by live animals as disease models -threatening.
Keywords: LaFora disease; cannabidiol; cannabinoids; epilepsy; neurodegeneration.

abnormal carbohydrate structure known as polyglucosan body (PGBs) associated with neurological disorders, glycogen storage disease (GSDs), and aging. A hallmark of the disease GSD LaFora (LD), a fatal epilepsy in children caused by recessive mutations in EPM2A or EPM2B gene, which PGBs cytoplasm known as LaFora body (LBS). LBS result from aberrant glycogen metabolism and disease progression drive. They are abundant in the brain, muscles and heart patients and Epm2a LD – / – and Epm2b – / – mice. LBS and histologically PGBs remind starch, carbohydrates semicrystalline synthesized for the storage of glucose in the plant.

In this study, we define the architecture LB, tissue-specific differences, and dynamics. We propose a model for how small polyglucosans aggregate to form LBS. LBS is very similar to PGBs aging and other neurological disorders, so this research has direct relevance to the general understanding of the structure and formation PGB.

disease LaFora

Progressive myoclonic epilepsies (PMEs) is a rare genetic disorder of various age groups (infants, children, youth, or adult-onset) is characterized by progressive myoclonus, epileptic seizures and in most cases, dementia and ataxia. Death is the worst possible outcome.

Genetic research has led to the identification of many genes culprit, and others are expected to be found. PMEs including disease Unverricht-Lundborg (ULD), disease LaFora (LD), lipofuscinoses ceroid neuronal, sialidosis type I, epilepsy myoclonus and fiber red ragged (MERRF), Gaucher disease type 3, dentatorubral-pallidoluysian atrophy (DRPLA), and other rare forms of PMEs.

ALV Real-Time PCR Kit
PD65-15	Bionote	96t	EUR 1724.4
Description: Please check the datasheet of ALV Real-Time PCR Kit before using the test.

Human Real-time PCR DNA Quantitation after QPCR DNA Damage Analysis Kit
DD2H	Detroit R&D	1 Kit	EUR 430

Mouse Real-time PCR DNA Quantitation after QPCR DNA Damage Analysis Kit
DD2M	Detroit R&D	1 Kit	EUR 430

Rat Real-time PCR DNA Quantitation after QPCR DNA Damage Analysis Kit
DD2R	Detroit R&D	1 Kit	EUR 430

Eztime Real - time PCRPremix 2x,Taqman
FYT105-100P	Yeastern Biotech	100 Preps 1.25 ml	Ask for price

Eztime Real - time PCRPremix 2x,SYBR Green
FYT103-100P	Yeastern Biotech	100 Preps 1.25 ml	Ask for price

Eztime Real - time PCRPremix 2x,SYBR Green
FYT103-400P	Yeastern Biotech	400 Preps 1.25 ml x 4	Ask for price

Eztime Real - time PCRPremix 2x,SYBRGreen , ROX
FYT104-100P	Yeastern Biotech	100 Preps 1.25 ml	Ask for price

Eztime Real - time PCRPremix 2x,SYBRGreen , ROX
FYT104-400P	Yeastern Biotech	400 Preps 1.25 ml x 4	Ask for price

Eztime Real - time PCRPremix 2x,Taqman, ROX
FYT105-400P	Yeastern Biotech	400 Preps 1.25 ml x 4	Ask for price

Eztime Real - time PCRPremix 2x,Taqman, ROX
FYT106-100P	Yeastern Biotech	100 Preps 1.25 ml	Ask for price

Human DNA Repair Primer Library I
HDRL-I	Real Time Primers	1 set	EUR 548

Human DNA Repair Primer Library II
HDRL-II	Real Time Primers	1 set	EUR 548

Mouse DNA Damage Signaling Primer Library
MDDS-I	Real Time Primers	1 set	EUR 548

Mouse DNA Repair Primer Library I
MDRL-I	Real Time Primers	1 set	EUR 645

Mouse DNA Repair Primer Library II
MDRL-II	Real Time Primers	1 set	EUR 645

Real Thiol
HY-108715	MedChemExpress	1mg	EUR 452

hsa-mir-521 Real-time RT-PCR Detection Kit
20-abx097600	Abbexa	EUR 551.00 EUR 787.00 EUR 398.00	100 rxns 200 rxns 50 rxns
Shipped within 3 weeks.

Azura PureView 250bp DNA Ladder - 100 Lanes
AZ-1121	Real Time Primers	100 Lanes	EUR 117

Azura PureView 250bp DNA Ladder - 500 Lanes
AZ-1125	Real Time Primers	500 Lanes	EUR 231

Azura PureView 250bp DNA Ladder - 1000 Lanes
AZ-1125-2	Real Time Primers	1000 Lanes	EUR 272

Azura PureView 100bp DNA Ladder - 100 Lanes
AZ-1131	Real Time Primers	100 Lanes	EUR 117

Azura PureView 100bp DNA Ladder - 500 Lanes
AZ-1135	Real Time Primers	500 Lanes	EUR 231

Azura PureView 100bp DNA Ladder - 1000 Lanes
AZ-1135-2	Real Time Primers	1000 Lanes	EUR 272

Azura PureView 50bp DNA Ladder - 100 Lanes
AZ-1151	Real Time Primers	100 Lanes	EUR 117

Azura PureView 50bp DNA Ladder - 500 Lanes
AZ-1155	Real Time Primers	500 Lanes	EUR 231

Azura PureView 50bp DNA Ladder - 1000 Lanes
AZ-1155-2	Real Time Primers	1000 Lanes	EUR 272

dsGreen for Real-Time PCR, 100×, 50 mL
91010	lumiprobe	50 mL	EUR 3169

dsGreen for Real-Time PCR, 100×, 10 mL
81010	lumiprobe	10 mL	EUR 869

dsGreen for Real-Time PCR, 100×, 5 mL
71010	lumiprobe	5 mL	EUR 625

dsGreen for Real-Time PCR, 100×, 1.5 mL
51010	lumiprobe	1.5 mL	EUR 331

dsGreen for Real-Time PCR, 100×, 1 mL
41010	lumiprobe	1 mL	EUR 249

dsGreen for Real-Time PCR, 100×, 100 uL
11010	lumiprobe	100 uL	EUR 59

dsGreen for Real-Time PCR, 100×, 2 mL
61010	lumiprobe	2 mL	EUR 363

0.2ML CLEAR STRIP CAPS FOR REAL TIME PCR
PCR-2CP-RT-C	CORNING	125/pk	EUR 223
Description: PCR Plates & Tubes; PCR Strip Tubes and Strip Caps - Axygen

Azura PureView 1 Kb DNA Ladder - 100 Lanes
AZ-1101	Real Time Primers	100 Lanes	EUR 117

Azura PureView 1 Kb DNA Ladder - 500 Lanes
AZ-1105	Real Time Primers	500 Lanes	EUR 231

Azura PureView 1 Kb DNA Ladder - 1000 Lanes
AZ-1105-2	Real Time Primers	1000 Lanes	EUR 272

PMA Real-Time PCR Bacterial Viability Kit - Salmonella enterica (invA)
31033	Biotium	1kit	EUR 428
Description: Minimum order quantity: 1 unit of 1kit

PMA Real-Time PCR Bacterial Viability Kit - Mycobacterium tuberculosis (groEL2)
31034	Biotium	1kit	EUR 399
Description: Minimum order quantity: 1 unit of 1kit

PMA Real-Time PCR Bacterial Viability Kit - Staphylococcus aureus (nuc)
31035	Biotium	1kit	EUR 399
Description: Minimum order quantity: 1 unit of 1kit

PMA Real-Time PCR Bacterial Viability Kit - Staphylococcus aureus (mecA)
31036	Biotium	1kit	EUR 399
Description: Minimum order quantity: 1 unit of 1kit

PMA Real-Time PCR Bacterial Viability Kit - E. coli (uidA)
31050	Biotium	1kit	EUR 428
Description: Minimum order quantity: 1 unit of 1kit

PMA Real-Time PCR Bacterial Viability Kit - Listeria monocytogenes (hly)
31051	Biotium	1kit	EUR 399
Description: Minimum order quantity: 1 unit of 1kit

PMA Real-Time PCR Bacterial Viability Kit - Legionella pneumophila (mip)
31053	Biotium	1kit	EUR 428
Description: Minimum order quantity: 1 unit of 1kit

PMA Real-Time PCR Bacterial Viability Kit - Salmonella enterica (invA) PMAxx
31033-X	Biotium	1kit	EUR 428
Description: Minimum order quantity: 1 unit of 1kit

PMA Real-Time PCR Bacterial Viability Kit - E. coli (uidA) PMAxx
31050-X	Biotium	1kit	EUR 428
Description: Minimum order quantity: 1 unit of 1kit

PMA Real-Time PCR Bacterial Viability Kit - Listeria monocytogenes (hly) PMAxx
31051-X	Biotium	1kit	EUR 428
Description: Minimum order quantity: 1 unit of 1kit

Novel Coronavirus COVID-19 (2019-nCoV) Real Time RT-PCR Kit
RR-0478-02	Liferiver	25 tests/kit	EUR 991
For use with PE5700, MJ-Opticon & other single color systems, ABI7000, ABI7300, ABI7500, ABI7900, ABI StepOne, StepOne plus, MJ-Opticon2, MJ-chromo4, MX3000P, MX3005P, Smart Cycler II, Rotor-Gene 6000, LightCycler 480, CFX 96, Life 96, Slan 96, iCycl Show more
Description: Novel Coronavirus (2019-nCoV) Real Time RT-PCR Kit is used for the qualitative detection of a novel coronavirus, which was identified in 2019 at Wuhan City, Hubei Province, China, in upper respiratory tract specimens (nasopharyngeal extracts, deep cough sputum, etc.) and lower respiratory tract specimens (alveoli irrigation fluid, etc.) by real time PCR systems.

Goat Brucellosis(Brucellosis) ELISA Kit
QY-E140053	Qayee Biotechnology	96T	EUR 413

Sheep Brucellosis(Brucellosis) ELISA Kit
QY-E140054	Qayee Biotechnology	96T	EUR 413

Complete Bacterial Protein Extraction Reagent
AR0157	BosterBio	50mL (sufficient for lysis of 10g of bacterial biomass)	EUR 141

hsa-mir-521 Real-Time RT-PCR Detection and U6 Calibration Kit
20-abx097601	Abbexa	EUR 732.00 EUR 1038.00 EUR 523.00	100 rxns 200 rxns 50 rxns
Shipped within 3 weeks.

PMA Real-Time PCR Bacterial Viability Kit - E. coli O157:H7 (Z3276)
31037	Biotium	1kit	EUR 428
Description: Minimum order quantity: 1 unit of 1kit

qPCR plate-foil universal (for Real-time PCR, Light Cycler 480)
A26979	GeneOn	1x100 pcs	EUR 186

Green Dye One Step qRT-PCR Master Mix For Quantitative Real Time PCR With ROX Dye
MB1004-100Reactions	BosterBio	100 Reactions	EUR 327

AzuraQuant cDNA Synthesis Kit - 25 Reactions
AZ-1995	Real Time Primers	25 Reactions	EUR 153

AzuraQuant cDNA Synthesis Kit - 100 Reactions
AZ-1996	Real Time Primers	100 Reactions	EUR 372

Total Protein Extraction Kit: TM Buffer
K3011010-1	Biochain	13 ml	EUR 162
Description: Can be used for various proteomics studies in both normal and pathological cases. It is an excellent control and suitable for educational purposes. This product is prepared from whole tissue homogenates and has undergone SDS-PAGE quality control analysis. The protein is stored in a buffer with protease inhibitor cocktail fo prevent degradation.

PMA Real-Time PCR Bacterial Viability Kit - E. coli O157:H7 (Z3276) PMAxx
31037-X	Biotium	1kit	EUR 428
Description: Minimum order quantity: 1 unit of 1kit

0,1ML THIN WALL TUBES 8 PER STRIP. CLEAR & REAL TIME STRIP CAPS
PCR-0108-LP-RT-C	CORNING	125/pk	EUR 709
Description: PCR Plates & Tubes; PCR Strip Tubes and Strip Caps - Axygen

0,1ML THIN WALL TUBES 8 PER STRIP. WHITE & REAL TIME STRIP CAPS
PCR-0108-LP-RT-W	CORNING	125/pk	EUR 770
Description: PCR Plates & Tubes; PCR Strip Tubes and Strip Caps - Axygen

Genomic DNA Extraction Kit
K5016005	Biochain	1 kit	EUR 329

Exosomal DNA Extraction Kit
P230-25	101Bio	NULL	EUR 0

Exosomal DNA Extraction Kit
P230-50	101Bio	NULL	EUR 0

AzuraView GreenFast qPCR Blue Mix Lo Rox - 200 Rxn
AZ-2301	Real Time Primers	200 Rxn	EUR 179

AzuraView GreenFast qPCR Blue Mix Lo Rox - 500 Rxn
AZ-2305	Real Time Primers	500 Rxn	EUR 320

AzuraView GreenFast qPCR Blue Mix Lo Rox - 2000 Rxn
AZ-2320	Real Time Primers	2000 Rxn	EUR 996

AzuraView GreenFast qPCR Blue Mix Lo Rox - 5000 Rxn
AZ-2325	Real Time Primers	5000 Rxn	EUR 2348

AzuraView GreenFast qPCR Blue Mix Hi Rox - 200 Rxn
AZ-2401	Real Time Primers	200 Rxn	EUR 179

AzuraView GreenFast qPCR Blue Mix Hi Rox - 500 Rxn
AZ-2405	Real Time Primers	500 Rxn	EUR 320

AzuraView GreenFast qPCR Blue Mix Hi Rox - 2000 Rxn
AZ-2420	Real Time Primers	2000 Rxn	EUR 996

AzuraView GreenFast qPCR Blue Mix Hi Rox - 5000 Rxn
AZ-2421	Real Time Primers	5000 Rxn	EUR 2348

Human Endothelial Cell Primer Library
HEND-I	Real Time Primers	1 set	EUR 548

Human ERBB Signaling Primer Library
HERBB-I	Real Time Primers	1 set	EUR 548

Human Estrogen Signaling Primer Library
HESR-I	Real Time Primers	1 set	EUR 645

Human Focal Adhesion Signaling Primer Library
HFCA-I	Real Time Primers	1 set	EUR 548

Human FGF Receptor Signaling Primer Library
HFGF-I	Real Time Primers	1 set	EUR 645

Human Fatty Acid and Lipid Metabolism Primer Library
HFLM-I	Real Time Primers	1 set	EUR 548

Human Gastric Cancer Primer Library
HGCPL-I	Real Time Primers	1 set	EUR 548

Human GDNF Signaling Primer Library
HGDNF-I	Real Time Primers	1 set	EUR 548

Human Glucose Metabolism Primer Library
HGLM-I	Real Time Primers	1 set	EUR 450

Human G-Protein Coupled Receptor Signaling Primer Library
HGPCR-I	Real Time Primers	1 set	EUR 548

Human Hedgehog Signaling Primer Library
HHOG-I	Real Time Primers	1 set	EUR 645

Human Homeobox Gene Primer Library
HHOX-I	Real Time Primers	1 set	EUR 645

Human Hippo Signaling Primer Pathway
HHPO-I	Real Time Primers	1 set	EUR 548

Human Heat Shock Protein Primer Library
HHSP-I	Real Time Primers	1 set	EUR 743

Human Hypoxia Signaling Primer Library
HHYP-I	Real Time Primers	1 set	EUR 548

Human Interferon Type I Signaling Primer Library
HIFN-I	Real Time Primers	1 set	EUR 548

Human Interferon Type II Signaling Primer Library
HIFN-II	Real Time Primers	1 set	EUR 548

Human IL17 Signaling Primer Library
HIL17-I	Real Time Primers	1 set	EUR 548

Human Insulin Signaling Primer Library
HINS-I	Real Time Primers	1 set	EUR 548

Human Integrin Signaling Primer Library
HINT-I	Real Time Primers	1 set	EUR 548

Human JAK/STAT Signaling Primer Library
HJAK-I	Real Time Primers	1 set	EUR 548

Human Kinase Library I
HKIN-I	Real Time Primers	1 set	EUR 450

Human Kinase Library II
HKIN-II	Real Time Primers	1 set	EUR 450

Human Kinase Library III
HKIN-III	Real Time Primers	1 set	EUR 450

Human Kinase Library IV
HKIN-IV	Real Time Primers	1 set	EUR 450

Human Kinase Library (I, II, III and IV)
HKIN-X	Real Time Primers	1 set	EUR 1425

Human Liver Cancer Primer Library
HLCPL-I	Real Time Primers	1 set	EUR 548

Human Ligand Gated Ion Channels Primer Library
HLGI-I	Real Time Primers	1 set	EUR 743

Human Lung Cancer PCR Primer Library
HLUCPL-I	Real Time Primers	1 set	EUR 548

Human MAP Kinase Signaling Library
HMAP-I	Real Time Primers	1 set	EUR 548

Human Myeloid Derived Suppressor Cell Primer Library
HMDSC-I	Real Time Primers	1 set	EUR 548

Human Melanoma Primer Library
HMEL-I	Real Time Primers	1 set	EUR 548

Human Necroptosis Primer Library
HNCR-I	Real Time Primers	1 set	EUR 548

Human NFKappaB Primer Library
HNFKB-I	Real Time Primers	1 set	EUR 450

Human NK Cell Mediated Toxicity Primer Library
HNKT-I	Real Time Primers	1 set	EUR 548

Human Nitric Oxide Signaling Primer Library
HNOS-I	Real Time Primers	1 set	EUR 548

Human Notch Signaling Pathway
HNOT-I	Real Time Primers	1 set	EUR 548

Human Neurotransmitter Signaling Primer Library
HNTS-I	Real Time Primers	1 set	EUR 548

Human Oncogene Primer Library
HONC-I	Real Time Primers	1 set	EUR 548

Human Oxidative Stress Primer Library
HOSL-I	Real Time Primers	1 set	EUR 450

Human Osteogenesis Primer Library
HOST-I	Real Time Primers	1 set	EUR 548

Human Ovarian Cancer Primer Library
HOVCL-I	Real Time Primers	1 set	EUR 548

Human Apoptosis Primer Library
HPA-I	Real Time Primers	1 set	EUR 450

Human Pancreatic Cancer Primer Library
HPANC-I	Real Time Primers	1 set	EUR 548

Human PDGF Signaling Primer Library
HPDGF-I	Real Time Primers	1 set	EUR 548

Human Parkinson's Disease Primer Library
HPKD-I	Real Time Primers	1 set	EUR 548

Human Prostate Cancer Primer Library
HPRC-I	Real Time Primers	1 set	EUR 548

Human Ras Signaling Primer Library
HRAS-I	Real Time Primers	1 set	EUR 548

Human Retinoblastoma Gene Primer Library
HRBG-I	Real Time Primers	1 set	EUR 548

Human Rho GTPase-Signaling Primer Library
HRGTP-I	Real Time Primers	1 set	EUR 645

Human Schwann Cell PCR Primer Library
HSCH-I	Real Time Primers	1 set	EUR 548

Human Cytoskeleton Primer Library
HSCK-I	Real Time Primers	1 set	EUR 645

Human Stem Cell Primer Library
HSCL-I	Real Time Primers	1 set	EUR 450

Human SMAD Signaling Primer Library
HSMAD-I	Real Time Primers	1 set	EUR 548

Human Statin Signaling Library
HSTAT-I	Real Time Primers	1 set	EUR 548

Human Signal Transduction Primer Library
HSTL-I	Real Time Primers	1 set	EUR 450

Human T-Cell Anergy/Immune Tolerance Primer Library
HTAIT-I	Real Time Primers	1 set	EUR 548

Human T-Cell Receptor Signaling Primer Library
HTCR-I	Real Time Primers	1 set	EUR 548

Human T-Cell Development and Differentiation Primer Library
HTDD-I	Real Time Primers	1 set	EUR 548

Human TGF Beta Signaling Primer Library
HTGFB-I	Real Time Primers	1 set	EUR 548

Human Th1 and Th2 Cell Differentiation Primer Library
HTHD-I	Real Time Primers	1 set	EUR 548

Human Tumor Invasion/Metastasis Primer Library
HTIM-I	Real Time Primers	1 set	EUR 450

Human Tumor Immunity Primer Library
HTIMU-I	Real Time Primers	1 set	EUR 548

Human Toll-like Receptor Signaling Primer Library
HTLR-I	Real Time Primers	1 set	EUR 548

Human TNF Signaling Primer Library
HTNF-I	Real Time Primers	1 set	EUR 548

Human p53 Signaling Primer Library
HTPS-I	Real Time Primers	1 set	EUR 450

Human VEGF Signaling Primer Library
HVEGF-I	Real Time Primers	1 set	EUR 548

Human WNT Signaling Primer Library
HWNT-I	Real Time Primers	1 set	EUR 548

Human EGFR Signaling Primer Library
HEGFR-I	Real Time Primers	1 set	EUR 548

Human Epithelial Mesenchymal Transition Primer Library
HEMT-I	Real Time Primers	1 set	EUR 548

Human Actin Cytoskeleton Signaling Primer Library
HACS-I	Real Time Primers	1 set	EUR 548

Human Adipogenesis Primer Library
HADG-I	Real Time Primers	1 set	EUR 548

Human Adaptive & Innate Immune Response Primer Library
HAIIR-I	Real Time Primers	1 set	EUR 548

Human PI3K-AKT Signaling Primer Library
HAKT-I	Real Time Primers	1 set	EUR 450

Human Amino Acid Metabolism Primer Library
HAMM-I	Real Time Primers	1 set	EUR 645

Human Androgen Signaling Primer Library
HAND-I	Real Time Primers	1 set	EUR 548

Human Angiogenesis Primer Library
HAPL-I	Real Time Primers	1 set	EUR 450

This article will review LaFora disease, autosomal recessive PME characterized by loud and photosensitive myoclonic seizures, drop attacks, ataxia, apraxia, cortical blindness, and rapidly progressive dementia. diagnosis requires the presence of pathognomic LaFora bodies (abnormal glycogen inclusions) in tissue biopsies in addition to the exclusion of other forms of PMEs.