Cannabidiol-Enriched Extract Reduced the Cognitive Impairment but Not the Epileptic Seizures in a Lafora Disease Animal Model

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.

 Cannabidiol-Enriched Extract Reduced the Cognitive Impairment but Not the Epileptic Seizures in a Lafora Disease Animal Model
Cannabidiol-Enriched Extract Reduced the Cognitive Impairment but Not the Epileptic Seizures in a Lafora Disease Animal Model

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 96t
EUR 1724.40
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 1 Kit
EUR 430.00

Mouse Real-time PCR DNA Quantitation after QPCR DNA Damage Analysis Kit

DD2M 1 Kit
EUR 430.00

Rat Real-time PCR DNA Quantitation after QPCR DNA Damage Analysis Kit

DD2R 1 Kit
EUR 430.00

Eztime Real - time PCRPremix 2x,Taqman

FYT105-100P 100 Preps 1.25 ml Ask for price

Eztime Real - time PCRPremix 2x,SYBR Green

FYT103-100P 100 Preps 1.25 ml Ask for price

Eztime Real - time PCRPremix 2x,SYBR Green

FYT103-400P 400 Preps 1.25 ml x 4 Ask for price

Eztime Real - time PCRPremix 2x,SYBRGreen , ROX

FYT104-100P 100 Preps 1.25 ml Ask for price

Eztime Real - time PCRPremix 2x,SYBRGreen , ROX

FYT104-400P 400 Preps 1.25 ml x 4 Ask for price

Eztime Real - time PCRPremix 2x,Taqman, ROX

FYT105-400P 400 Preps 1.25 ml x 4 Ask for price

Eztime Real - time PCRPremix 2x,Taqman, ROX

FYT106-100P 100 Preps 1.25 ml Ask for price

Human DNA Repair Primer Library I

HDRL-I 1 set
EUR 548.00

Human DNA Repair Primer Library II

HDRL-II 1 set
EUR 548.00

Mouse DNA Damage Signaling Primer Library

MDDS-I 1 set
EUR 548.00

Mouse DNA Repair Primer Library I

MDRL-I 1 set
EUR 645.00

Mouse DNA Repair Primer Library II

MDRL-II 1 set
EUR 645.00

Azura PureView 250bp DNA Ladder - 100 Lanes

AZ-1121 100 Lanes
EUR 117.00

Azura PureView 250bp DNA Ladder - 500 Lanes

AZ-1125 500 Lanes
EUR 231.00

Azura PureView 250bp DNA Ladder - 1000 Lanes

AZ-1125-2 1000 Lanes
EUR 272.00

Azura PureView 100bp DNA Ladder - 100 Lanes

AZ-1131 100 Lanes
EUR 117.00

Azura PureView 100bp DNA Ladder - 500 Lanes

AZ-1135 500 Lanes
EUR 231.00

Azura PureView 100bp DNA Ladder - 1000 Lanes

AZ-1135-2 1000 Lanes
EUR 272.00

Azura PureView 50bp DNA Ladder - 100 Lanes

AZ-1151 100 Lanes
EUR 117.00

Azura PureView 50bp DNA Ladder - 500 Lanes

AZ-1155 500 Lanes
EUR 231.00

Azura PureView 50bp DNA Ladder - 1000 Lanes

AZ-1155-2 1000 Lanes
EUR 272.00

Real Thiol

HY-108715 1mg
EUR 452.00

Azura PureView 1 Kb DNA Ladder - 100 Lanes

AZ-1101 100 Lanes
EUR 117.00

Azura PureView 1 Kb DNA Ladder - 500 Lanes

AZ-1105 500 Lanes
EUR 231.00

Azura PureView 1 Kb DNA Ladder - 1000 Lanes

AZ-1105-2 1000 Lanes
EUR 272.00

dsGreen for Real-Time PCR, 100×, 5 mL

71010 5 mL
EUR 625.00

dsGreen for Real-Time PCR, 100×, 10 mL

81010 10 mL
EUR 869.00

dsGreen for Real-Time PCR, 100×, 50 mL

91010 50 mL
EUR 3169.00

dsGreen for Real-Time PCR, 100×, 2 mL

61010 2 mL
EUR 363.00

dsGreen for Real-Time PCR, 100×, 1.5 mL

51010 1.5 mL
EUR 331.00

dsGreen for Real-Time PCR, 100×, 100 uL

11010 100 uL
EUR 59.00

dsGreen for Real-Time PCR, 100×, 1 mL

41010 1 mL
EUR 249.00

0.2ML CLEAR STRIP CAPS FOR REAL TIME PCR

PCR-2CP-RT-C 125/pk
EUR 223.00
Description: PCR Plates & Tubes; PCR Strip Tubes and Strip Caps - Axygen

hsa-mir-521 Real-time RT-PCR Detection Kit

20-abx097600
  • EUR 551.00
  • EUR 787.00
  • EUR 398.00
  • 100 rxns
  • 200 rxns
  • 50 rxns
  • Shipped within 3 weeks.

PMA Real-Time PCR Bacterial Viability Kit - Salmonella enterica (invA)

31033 1kit
EUR 428.00
Description: Minimum order quantity: 1 unit of 1kit

PMA Real-Time PCR Bacterial Viability Kit - Mycobacterium tuberculosis (groEL2)

31034 1kit
EUR 399.00
Description: Minimum order quantity: 1 unit of 1kit

PMA Real-Time PCR Bacterial Viability Kit - Staphylococcus aureus (nuc)

31035 1kit
EUR 399.00
Description: Minimum order quantity: 1 unit of 1kit

PMA Real-Time PCR Bacterial Viability Kit - Staphylococcus aureus (mecA)

31036 1kit
EUR 399.00
Description: Minimum order quantity: 1 unit of 1kit

PMA Real-Time PCR Bacterial Viability Kit - E. coli (uidA)

31050 1kit
EUR 428.00
Description: Minimum order quantity: 1 unit of 1kit

PMA Real-Time PCR Bacterial Viability Kit - Listeria monocytogenes (hly)

31051 1kit
EUR 399.00
Description: Minimum order quantity: 1 unit of 1kit

PMA Real-Time PCR Bacterial Viability Kit - Legionella pneumophila (mip)

31053 1kit
EUR 428.00
Description: Minimum order quantity: 1 unit of 1kit

PMA Real-Time PCR Bacterial Viability Kit - Salmonella enterica (invA) PMAxx

31033-X 1kit
EUR 428.00
Description: Minimum order quantity: 1 unit of 1kit

PMA Real-Time PCR Bacterial Viability Kit - E. coli (uidA) PMAxx

31050-X 1kit
EUR 428.00
Description: Minimum order quantity: 1 unit of 1kit

PMA Real-Time PCR Bacterial Viability Kit - Listeria monocytogenes (hly) PMAxx

31051-X 1kit
EUR 428.00
Description: Minimum order quantity: 1 unit of 1kit

Novel Coronavirus COVID-19 (2019-nCoV) Real Time RT-PCR Kit

RR-0478-02 25 tests/kit
EUR 991.00
  • 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.

Complete Bacterial Protein Extraction Reagent

AR0157 50mL (sufficient for lysis of 10g of bacterial biomass)
EUR 141.00

Goat Brucellosis(Brucellosis) ELISA Kit

QY-E140053 96T
EUR 413.00

Sheep Brucellosis(Brucellosis) ELISA Kit

QY-E140054 96T
EUR 413.00

PMA Real-Time PCR Bacterial Viability Kit - E. coli O157:H7 (Z3276)

31037 1kit
EUR 428.00
Description: Minimum order quantity: 1 unit of 1kit

hsa-mir-521 Real-Time RT-PCR Detection and U6 Calibration Kit

20-abx097601
  • EUR 732.00
  • EUR 1038.00
  • EUR 523.00
  • 100 rxns
  • 200 rxns
  • 50 rxns
  • Shipped within 3 weeks.

qPCR plate-foil universal (for Real-time PCR, Light Cycler 480)

A26979 1x100 pcs
EUR 186.00

AzuraQuant cDNA Synthesis Kit - 25 Reactions

AZ-1995 25 Reactions
EUR 153.00

AzuraQuant cDNA Synthesis Kit - 100 Reactions

AZ-1996 100 Reactions
EUR 372.00

Green Dye One Step qRT-PCR Master Mix For Quantitative Real Time PCR With ROX Dye

MB1004-100Reactions 100 Reactions
EUR 327.00

PMA Real-Time PCR Bacterial Viability Kit - E. coli O157:H7 (Z3276) PMAxx

31037-X 1kit
EUR 428.00
Description: Minimum order quantity: 1 unit of 1kit

Total Protein Extraction Kit: TM Buffer

K3011010-1 13 ml
EUR 162.00
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.

0,1ML THIN WALL TUBES 8 PER STRIP. CLEAR & REAL TIME STRIP CAPS

PCR-0108-LP-RT-C 125/pk
EUR 709.00
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 125/pk
EUR 770.00
Description: PCR Plates & Tubes; PCR Strip Tubes and Strip Caps - Axygen

Exosomal DNA Extraction Kit

P230-25 NULL
EUR NULL

Exosomal DNA Extraction Kit

P230-50 NULL
EUR NULL

Genomic DNA Extraction Kit

K5016005 1 kit
EUR 329.00

AzuraView GreenFast qPCR Blue Mix Lo Rox - 200 Rxn

AZ-2301 200 Rxn
EUR 179.00

AzuraView GreenFast qPCR Blue Mix Lo Rox - 500 Rxn

AZ-2305 500 Rxn
EUR 320.00

AzuraView GreenFast qPCR Blue Mix Lo Rox - 2000 Rxn

AZ-2320 2000 Rxn
EUR 996.00

AzuraView GreenFast qPCR Blue Mix Lo Rox - 5000 Rxn

AZ-2325 5000 Rxn
EUR 2348.00

AzuraView GreenFast qPCR Blue Mix Hi Rox - 200 Rxn

AZ-2401 200 Rxn
EUR 179.00

AzuraView GreenFast qPCR Blue Mix Hi Rox - 500 Rxn

AZ-2405 500 Rxn
EUR 320.00

AzuraView GreenFast qPCR Blue Mix Hi Rox - 2000 Rxn

AZ-2420 2000 Rxn
EUR 996.00

AzuraView GreenFast qPCR Blue Mix Hi Rox - 5000 Rxn

AZ-2421 5000 Rxn
EUR 2348.00

Human Actin Cytoskeleton Signaling Primer Library

HACS-I 1 set
EUR 548.00

Human Adipogenesis Primer Library

HADG-I 1 set
EUR 548.00

Human Adaptive & Innate Immune Response Primer Library

HAIIR-I 1 set
EUR 548.00

Human PI3K-AKT Signaling Primer Library

HAKT-I 1 set
EUR 450.00

Human Amino Acid Metabolism Primer Library

HAMM-I 1 set
EUR 645.00

Human Androgen Signaling Primer Library

HAND-I 1 set
EUR 548.00

Human Angiogenesis Primer Library

HAPL-I 1 set
EUR 450.00

Human Antigen Processing and Presentation Primer Library

HAPP-I 1 set
EUR 548.00

Human Atherosclerosis Primer Library

HATH-I 1 set
EUR 548.00

Human Adherin - Tight Junction Primer Library

HATJ-I 1 set
EUR 548.00

Human Autophagy Primer Library

HATPL-I 1 set
EUR 450.00

Human Alzheimer's Disease Primer Library

HAZD-I 1 set
EUR 548.00

Human B-Cell Receptor Signaling Primer Library

HBCR-I 1 set
EUR 548.00

Human BDNF Signaling Primer Library

HBDNF-I 1 set
EUR 548.00

Human Bladder Cancer Primer Library

HBLCP-I 1 set
EUR 548.00

Human BMP Signaling Primer Library

HBMP-I 1 set
EUR 548.00

Human Brain Cancer Primer Library

HBRC-I 1 set
EUR 548.00

Human Breast Cancer Signaling Primer Library

HBRCA-I 1 set
EUR 548.00

Human Cytokine Primer Library I

HCA-I 1 set
EUR 450.00

Human Cytokine Primer Library II

HCA-II 1 set
EUR 450.00

Human Cell Adhesion Primer Library

HCAD-I 1 set
EUR 645.00

Human cAMP Signaling Primer Library

HCAMP-I 1 set
EUR 548.00

Human Cancer Pathway Primer Library

HCAN-I 1 set
EUR 548.00

Human Calcium Regulation Pathway

HCAR-I 1 set
EUR 548.00

Human Cell Cycle Primer Library

HCC-I 1 set
EUR 450.00

Human Colon Cancer Primer Library I

HCCP-I 1 set
EUR 548.00

Human Colon Cancer Primer Library II

HCCP-II 1 set
EUR 548.00

Human Cytokine and Chemokine Receptor Primer Library

HCCR-I 1 set
EUR 645.00

Human Cancer Driver Gene I Primer Library

HCDG-I 1 set
EUR 645.00

Human Cancer Driver Gene II Primer Library

HCDG-II 1 set
EUR 645.00

Human Chromatin Regulation Primer Library

HCHR-I 1 set
EUR 645.00

Human Oncogene circRNA Library

HCirc-Onc-I 1 set
EUR 938.00

Human CREB Signaling Primer Library

HCREB-I 1 set
EUR 548.00

Human Drug Transporter I Primer Library

HDTP-I 1 set
EUR 548.00

Human Drug Detoxication I

HDTX-I 1 set
EUR 548.00

Human Drug Detoxication II

HDTX-II 1 set
EUR 645.00

Human EGFR Signaling Primer Library

HEGFR-I 1 set
EUR 548.00

Human Epithelial Mesenchymal Transition Primer Library

HEMT-I 1 set
EUR 548.00

Human Endothelial Cell Primer Library

HEND-I 1 set
EUR 548.00

Human ERBB Signaling Primer Library

HERBB-I 1 set
EUR 548.00

Human Estrogen Signaling Primer Library

HESR-I 1 set
EUR 645.00

Human Focal Adhesion Signaling Primer Library

HFCA-I 1 set
EUR 548.00

Human FGF Receptor Signaling Primer Library

HFGF-I 1 set
EUR 645.00

Human Fatty Acid and Lipid Metabolism Primer Library

HFLM-I 1 set
EUR 548.00

Mouse Adipogenesis Primer Library

MADG-I 1 set
EUR 548.00

Mouse PI3K-AKT Signaling Primer Library

MAKT-I 1 set
EUR 548.00

Mouse Angiogenesis Primer Library

MAPL-I 1 set
EUR 450.00

Mouse Adherin - Tight Junction Primer Library

MATJ-I 1 set
EUR 548.00

Mouse Autophagy Primer Library

MATPL-I 1 set
EUR 450.00

Mouse Bdnf Signaling Primer Library

MBDNF-I 1 set
EUR 548.00

Mouse BMP Signaling Primer Library

MBMP-I 1 set
EUR 548.00

Mouse Cytokine Primer Library I

MCA-I 1 set
EUR 450.00

Mouse Cytokine Primer Library II

MCA-II 1 set
EUR 450.00

Mouse Cell Cycle Primer Library

MCC-I 1 set
EUR 450.00

Mouse Cytokine and Chemokine Receptor Primer Library

MCCR-I 1 set
EUR 645.00

Mouse CREB Signaling Primer Library

MCREB-I 1 set
EUR 548.00

Mouse EGFR Signaling Primer Library

MEGFR-I 1 set
EUR 548.00

Mouse Epithelial-Mesenchymal Transition Primer Library

MEMT-I 1 set
EUR 548.00

Mouse Estrogen Receptor Signaling Library

MESR-I 1 set
EUR 645.00

Mouse Fatty Liver Signaling Primer Library

MFTL-I 1 set
EUR 548.00

Mouse Gdnf Signaling Primer Library

MGDNF-I 1 set
EUR 548.00

Mouse Glucose Metabolism Primer Library

MGLM-I 1 set
EUR 548.00

Mouse Hippo Signaling Primer Library

MHPO-I 1 set
EUR 548.00

Mouse Hypoxia Signaling Primer Library

MHYP-I 1 set
EUR 548.00

Human Gastric Cancer Primer Library

HGCPL-I 1 set
EUR 548.00

Human GDNF Signaling Primer Library

HGDNF-I 1 set
EUR 548.00

Human Glucose Metabolism Primer Library

HGLM-I 1 set
EUR 450.00

Human G-Protein Coupled Receptor Signaling Primer Library

HGPCR-I 1 set
EUR 548.00

Human Hedgehog Signaling Primer Library

HHOG-I 1 set
EUR 645.00

Human Homeobox Gene Primer Library

HHOX-I 1 set
EUR 645.00

Human Hippo Signaling Primer Pathway

HHPO-I 1 set
EUR 548.00

Human Heat Shock Protein Primer Library

HHSP-I 1 set
EUR 743.00

Human Hypoxia Signaling Primer Library

HHYP-I 1 set
EUR 548.00

Human Interferon Type I Signaling Primer Library

HIFN-I 1 set
EUR 548.00

Human Interferon Type II Signaling Primer Library

HIFN-II 1 set
EUR 548.00

Human IL17 Signaling Primer Library

HIL17-I 1 set
EUR 548.00

Human Insulin Signaling Primer Library

HINS-I 1 set
EUR 548.00

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.