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.
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.
Sheep Brucellosis(Brucellosis) ELISA Kit |
|||
QY-E140054 | Qayee Biotechnology | 96T | EUR 495.6 |
Complete Bacterial Protein Extraction Reagent |
|||
AR0157 | BosterBio | 50mL (sufficient for lysis of 10g of bacterial biomass) | EUR 169.2 |
Goat Brucellosis ELISA Kit |
|||
SL0083Gt | Sunlong | - | Ask for price |
Sheep Brucellosis ELISA Kit |
|||
SL00139Sp | Sunlong | - | Ask for price |
Human Brucellosis ELISA Kit |
|||
SL3758Hu | Sunlong | 96 Tests | EUR 468 |
Canine Brucellosis ELISA Kit |
|||
SL0094Ca | Sunlong | - | Ask for price |
Goat brucellosis antibody ELISA Kit |
|||
ELA-E2191g | Lifescience Market | 96 Tests | EUR 1113.6 |
AzuraQuant cDNA Synthesis Kit - 100 Reactions |
|||
AZ-1996 | Real Time Primers | 100 Reactions | EUR 446.4 |
Mouse DNA Repair Primer Library I |
|||
MDRL-I | Real Time Primers | 1 set | EUR 774 |
Mouse DNA Repair Primer Library II |
|||
MDRL-II | Real Time Primers | 1 set | EUR 774 |
Human DNA Repair Primer Library I |
|||
HDRL-I | Real Time Primers | 1 set | EUR 657.6 |
Human DNA Repair Primer Library II |
|||
HDRL-II | Real Time Primers | 1 set | EUR 657.6 |
Mouse DNA Damage Signaling Primer Library |
|||
MDDS-I | Real Time Primers | 1 set | EUR 657.6 |
Azura PureView 250bp DNA Ladder - 100 Lanes |
|||
AZ-1121 | Real Time Primers | 100 Lanes | EUR 140.4 |
Azura PureView 250bp DNA Ladder - 500 Lanes |
|||
AZ-1125 | Real Time Primers | 500 Lanes | EUR 277.2 |
Azura PureView 250bp DNA Ladder - 1000 Lanes |
|||
AZ-1125-2 | Real Time Primers | 1000 Lanes | EUR 326.4 |
Azura PureView 100bp DNA Ladder - 100 Lanes |
|||
AZ-1131 | Real Time Primers | 100 Lanes | EUR 140.4 |
Azura PureView 100bp DNA Ladder - 500 Lanes |
|||
AZ-1135 | Real Time Primers | 500 Lanes | EUR 277.2 |
Azura PureView 100bp DNA Ladder - 1000 Lanes |
|||
AZ-1135-2 | Real Time Primers | 1000 Lanes | EUR 326.4 |
Azura PureView 50bp DNA Ladder - 100 Lanes |
|||
AZ-1151 | Real Time Primers | 100 Lanes | EUR 140.4 |
Azura PureView 50bp DNA Ladder - 500 Lanes |
|||
AZ-1155 | Real Time Primers | 500 Lanes | EUR 277.2 |
Azura PureView 50bp DNA Ladder - 1000 Lanes |
|||
AZ-1155-2 | Real Time Primers | 1000 Lanes | EUR 326.4 |
Azura PureView 1 Kb DNA Ladder - 100 Lanes |
|||
AZ-1101 | Real Time Primers | 100 Lanes | EUR 140.4 |
Azura PureView 1 Kb DNA Ladder - 500 Lanes |
|||
AZ-1105 | Real Time Primers | 500 Lanes | EUR 277.2 |
Azura PureView 1 Kb DNA Ladder - 1000 Lanes |
|||
AZ-1105-2 | Real Time Primers | 1000 Lanes | EUR 326.4 |
AzuraQuant cDNA Synthesis Kit - 25 Reactions |
|||
AZ-1995 | Real Time Primers | 25 Reactions | EUR 183.6 |
Minute TM Histon/DNA Binding Protein Extraction Kit (50 Tests) |
|||
HP-014 | Inventbiotech | each | EUR 399 |
Truenat™ Chip-based Real Time PCR Test CT |
|||
CT | Molbio Diagnostics Private Limited | each | EUR 18 |
Truenat™ Chip-based Real Time PCR Test NG |
|||
NG | Molbio Diagnostics Private Limited | each | EUR 18 |
Truenat™ Chip-based Real Time PCR Test GBS |
|||
GBS | Molbio Diagnostics Private Limited | each | EUR 18 |
Truenat™ Chip-based Real Time PCR Test HAV |
|||
HAV | Molbio Diagnostics Private Limited | each | EUR 18 |
Truenat™ Chip-based Real Time PCR Test HBV |
|||
HBV | Molbio Diagnostics Private Limited | each | EUR 16 |
Truenat™ Chip-based Real Time PCR Test HCV |
|||
HCV | Molbio Diagnostics Private Limited | each | EUR 18 |
Truenat™ Chip-based Real Time PCR Test HEV |
|||
HEV | Molbio Diagnostics Private Limited | each | EUR 18 |
Truenat™ Chip-based Real Time PCR Test HPV |
|||
HPV | Molbio Diagnostics Private Limited | each | EUR 20 |
Truenat™ Chip-based Real Time PCR Test MTB |
|||
MTB | Molbio Diagnostics Private Limited | each | EUR 12 |
Truenat™ Chip-based Real Time PCR Test H1N1 |
|||
H1N1 | Molbio Diagnostics Private Limited | each | EUR 18 |
Truenat™ Chip-based Real Time PCR Test Trich |
|||
Trich | Molbio Diagnostics Private Limited | each | EUR 18 |
Truenat™ Chip-based Real Time PCR Test Dengue |
|||
Dengue | Molbio Diagnostics Private Limited | each | EUR 18 |
Truenat™ Chip-based Real Time PCR Test Rabies |
|||
Rabies | Molbio Diagnostics Private Limited | each | EUR 18 |
Truenat™ Chip-based Real Time PCR Test Chikungunya |
|||
Chikungunya | Molbio Diagnostics Private Limited | each | EUR 18 |
Truenat™ Chip-based Real Time PCR Test CT/NG |
|||
CT/NG | Molbio Diagnostics Private Limited | each | EUR 20 |
FFPE DNA Extraction Kit |
|||
EP016 | ELK Biotech | 50T | EUR 140 |
Cell DNA Extraction Kit |
|||
TBS6007 | Tribioscience | 1000 samples | EUR 258 |
VIRAL DNA EXTRACTION KIT |
|||
IB47400 | IBI Scientific | 4 PREPS | EUR 30.11 |
VIRAL DNA EXTRACTION KIT |
|||
IB47401 | IBI Scientific | 50 PREPS | EUR 238.33 |
VIRAL DNA EXTRACTION KIT |
|||
IB47402 | IBI Scientific | 100 PREPS | EUR 428.75 |
VIRAL DNA EXTRACTION KIT |
|||
IB47403 | IBI Scientific | 300 PREPS | EUR 1151.98 |
Blood DNA Extraction Kit |
|||
TBS6004 | Tribioscience | 1000 samples | EUR 298 |
Truenat™ Chip-based Real Time PCR Test MTB RIF |
|||
MTB_RIF | Molbio Diagnostics Private Limited | each | EUR 9 |
Truenat™ Chip-based Real Time PCR Test MTB Plus |
|||
MTB_Plus | Molbio Diagnostics Private Limited | each | EUR 12 |
AzuraView GreenFast qPCR Blue Mix Lo Rox - 200 Rxn |
|||
AZ-2301 | Real Time Primers | 200 Rxn | EUR 214.8 |
AzuraView GreenFast qPCR Blue Mix Lo Rox - 500 Rxn |
|||
AZ-2305 | Real Time Primers | 500 Rxn | EUR 384 |
AzuraView GreenFast qPCR Blue Mix Lo Rox - 2000 Rxn |
|||
AZ-2320 | Real Time Primers | 2000 Rxn | EUR 1195.2 |
AzuraView GreenFast qPCR Blue Mix Lo Rox - 5000 Rxn |
|||
AZ-2325 | Real Time Primers | 5000 Rxn | EUR 2817.6 |
AzuraView GreenFast qPCR Blue Mix Hi Rox - 200 Rxn |
|||
AZ-2401 | Real Time Primers | 200 Rxn | EUR 214.8 |
AzuraView GreenFast qPCR Blue Mix Hi Rox - 500 Rxn |
|||
AZ-2405 | Real Time Primers | 500 Rxn | EUR 384 |
AzuraView GreenFast qPCR Blue Mix Hi Rox - 2000 Rxn |
|||
AZ-2420 | Real Time Primers | 2000 Rxn | EUR 1195.2 |
AzuraView GreenFast qPCR Blue Mix Hi Rox - 5000 Rxn |
|||
AZ-2421 | Real Time Primers | 5000 Rxn | EUR 2817.6 |
Mouse Gdnf Signaling Primer Library |
|||
MGDNF-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse Glucose Metabolism Primer Library |
|||
MGLM-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse Hippo Signaling Primer Library |
|||
MHPO-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse Hypoxia Signaling Primer Library |
|||
MHYP-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse Notch Signaling Primer Library |
|||
MNOT-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse Neurotransmitter Signaling Primer Library |
|||
MNTS-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse Integrin Signaling Primer Library |
|||
MINT-I | Real Time Primers | 1 set | EUR 891.6 |
Mouse Oxidative Stress Primer Library |
|||
MOSL-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse Oncogene Primer Library |
|||
MONC-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse Apoptosis Primer Library |
|||
MPA-I | Real Time Primers | 1 set | EUR 540 |
Mouse Ras Signaling Primer Library |
|||
MRAS-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse T-Cell Receptor Signaling Primer Library |
|||
MTCR-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse TGF Beta Signaling Primer Library |
|||
MTGFB-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse Tumor Invasion/Metastasis Primer Library |
|||
MTIM-I | Real Time Primers | 1 set | EUR 540 |
Mouse Tumor Immunity Primer Library |
|||
MTIMU-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse p53 Signaling Primer Library |
|||
MTPS-I | Real Time Primers | 1 set | EUR 540 |
Mouse WNT Signaling Primer Library |
|||
MWNT-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse Toll-like Receptor Signaling Primer Library |
|||
newitem | Real Time Primers | 1 set | EUR 657.6 |
Mouse PI3K-AKT Signaling Primer Library |
|||
MAKT-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse Angiogenesis Primer Library |
|||
MAPL-I | Real Time Primers | 1 set | EUR 540 |
Mouse Adherin - Tight Junction Primer Library |
|||
MATJ-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse Autophagy Primer Library |
|||
MATPL-I | Real Time Primers | 1 set | EUR 540 |
Mouse Bdnf Signaling Primer Library |
|||
MBDNF-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse BMP Signaling Primer Library |
|||
MBMP-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse Cytokine Primer Library I |
|||
MCA-I | Real Time Primers | 1 set | EUR 540 |
Mouse Cytokine Primer Library II |
|||
MCA-II | Real Time Primers | 1 set | EUR 540 |
Mouse Cell Cycle Primer Library |
|||
MCC-I | Real Time Primers | 1 set | EUR 540 |
Mouse Cytokine and Chemokine Receptor Primer Library |
|||
MCCR-I | Real Time Primers | 1 set | EUR 774 |
Mouse CREB Signaling Primer Library |
|||
MCREB-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse Adipogenesis Primer Library |
|||
MADG-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse EGFR Signaling Primer Library |
|||
MEGFR-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse Epithelial-Mesenchymal Transition Primer Library |
|||
MEMT-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse Estrogen Receptor Signaling Library |
|||
MESR-I | Real Time Primers | 1 set | EUR 774 |
Mouse Fatty Liver Signaling Primer Library |
|||
MFTL-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse Jak/Stat Signaling Primer Library |
|||
MJAK-I | Real Time Primers | 1 set | EUR 774 |
Mouse MAP Kinase Signaling Primer Library |
|||
MMAP-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse Myeloid Derived Suppressor Cell Primer Library |
|||
MMDSC-I | Real Time Primers | 1 set | EUR 657.6 |
Mouse NFKappaB Primer Library |
|||
MNFKB-I | Real Time Primers | 1 set | EUR 540 |
Human Drug Transporter I Primer Library |
|||
HDTP-I | Real Time Primers | 1 set | EUR 657.6 |
Human Drug Detoxication I |
|||
HDTX-I | Real Time Primers | 1 set | EUR 657.6 |
Human Drug Detoxication II |
|||
HDTX-II | Real Time Primers | 1 set | EUR 774 |
Human EGFR Signaling Primer Library |
|||
HEGFR-I | Real Time Primers | 1 set | EUR 657.6 |
Human Epithelial Mesenchymal Transition Primer Library |
|||
HEMT-I | Real Time Primers | 1 set | EUR 657.6 |
Human Endothelial Cell Primer Library |
|||
HEND-I | Real Time Primers | 1 set | EUR 657.6 |
Human ERBB Signaling Primer Library |
|||
HERBB-I | Real Time Primers | 1 set | EUR 657.6 |
Human Estrogen Signaling Primer Library |
|||
HESR-I | Real Time Primers | 1 set | EUR 774 |
Human Focal Adhesion Signaling Primer Library |
|||
HFCA-I | Real Time Primers | 1 set | EUR 657.6 |
Human FGF Receptor Signaling Primer Library |
|||
HFGF-I | Real Time Primers | 1 set | EUR 774 |
Human Fatty Acid and Lipid Metabolism Primer Library |
|||
HFLM-I | Real Time Primers | 1 set | EUR 657.6 |
Human Gastric Cancer Primer Library |
|||
HGCPL-I | Real Time Primers | 1 set | EUR 657.6 |
Human GDNF Signaling Primer Library |
|||
HGDNF-I | Real Time Primers | 1 set | EUR 657.6 |
Human Glucose Metabolism Primer Library |
|||
HGLM-I | Real Time Primers | 1 set | EUR 540 |
Human G-Protein Coupled Receptor Signaling Primer Library |
|||
HGPCR-I | Real Time Primers | 1 set | EUR 657.6 |
Human Hedgehog Signaling Primer Library |
|||
HHOG-I | Real Time Primers | 1 set | EUR 774 |
Human Homeobox Gene Primer Library |
|||
HHOX-I | Real Time Primers | 1 set | EUR 774 |
Human Hippo Signaling Primer Pathway |
|||
HHPO-I | Real Time Primers | 1 set | EUR 657.6 |
Human Heat Shock Protein Primer Library |
|||
HHSP-I | Real Time Primers | 1 set | EUR 891.6 |
Human Hypoxia Signaling Primer Library |
|||
HHYP-I | Real Time Primers | 1 set | EUR 657.6 |
Human Interferon Type I Signaling Primer Library |
|||
HIFN-I | Real Time Primers | 1 set | EUR 657.6 |
Human Interferon Type II Signaling Primer Library |
|||
HIFN-II | Real Time Primers | 1 set | EUR 657.6 |
Human IL17 Signaling Primer Library |
|||
HIL17-I | Real Time Primers | 1 set | EUR 657.6 |
Human Insulin Signaling Primer Library |
|||
HINS-I | Real Time Primers | 1 set | EUR 657.6 |
Human Integrin Signaling Primer Library |
|||
HINT-I | Real Time Primers | 1 set | EUR 657.6 |
Human Actin Cytoskeleton Signaling Primer Library |
|||
HACS-I | Real Time Primers | 1 set | EUR 657.6 |
Human Adipogenesis Primer Library |
|||
HADG-I | Real Time Primers | 1 set | EUR 657.6 |
Human Adaptive & Innate Immune Response Primer Library |
|||
HAIIR-I | Real Time Primers | 1 set | EUR 657.6 |
Human PI3K-AKT Signaling Primer Library |
|||
HAKT-I | Real Time Primers | 1 set | EUR 540 |
Human Amino Acid Metabolism Primer Library |
|||
HAMM-I | Real Time Primers | 1 set | EUR 774 |
Human Androgen Signaling Primer Library |
|||
HAND-I | Real Time Primers | 1 set | EUR 657.6 |
Human Angiogenesis Primer Library |
|||
HAPL-I | Real Time Primers | 1 set | EUR 540 |
Human Antigen Processing and Presentation Primer Library |
|||
HAPP-I | Real Time Primers | 1 set | EUR 657.6 |
Human Atherosclerosis Primer Library |
|||
HATH-I | Real Time Primers | 1 set | EUR 657.6 |
Human Adherin - Tight Junction Primer Library |
|||
HATJ-I | Real Time Primers | 1 set | EUR 657.6 |
Human Autophagy Primer Library |
|||
HATPL-I | Real Time Primers | 1 set | EUR 540 |
Human Alzheimer's Disease Primer Library |
|||
HAZD-I | Real Time Primers | 1 set | EUR 657.6 |
Human B-Cell Receptor Signaling Primer Library |
|||
HBCR-I | Real Time Primers | 1 set | EUR 657.6 |
Human BDNF Signaling Primer Library |
|||
HBDNF-I | Real Time Primers | 1 set | EUR 657.6 |
Human Bladder Cancer Primer Library |
|||
HBLCP-I | Real Time Primers | 1 set | EUR 657.6 |
Human BMP Signaling Primer Library |
|||
HBMP-I | Real Time Primers | 1 set | EUR 657.6 |
Human Brain Cancer Primer Library |
|||
HBRC-I | Real Time Primers | 1 set | EUR 657.6 |
Human Breast Cancer Signaling Primer Library |
|||
HBRCA-I | Real Time Primers | 1 set | EUR 657.6 |
Human Cytokine Primer Library I |
|||
HCA-I | Real Time Primers | 1 set | EUR 540 |
Human Cytokine Primer Library II |
|||
HCA-II | Real Time Primers | 1 set | EUR 540 |
Human Cell Adhesion Primer Library |
|||
HCAD-I | Real Time Primers | 1 set | EUR 774 |
Human cAMP Signaling Primer Library |
|||
HCAMP-I | Real Time Primers | 1 set | EUR 657.6 |
Human Cancer Pathway Primer Library |
|||
HCAN-I | Real Time Primers | 1 set | EUR 657.6 |
Human Calcium Regulation Pathway |
|||
HCAR-I | Real Time Primers | 1 set | EUR 657.6 |
Human Cell Cycle Primer Library |
|||
HCC-I | Real Time Primers | 1 set | EUR 540 |
Human Colon Cancer Primer Library I |
|||
HCCP-I | Real Time Primers | 1 set | EUR 657.6 |
Human Colon Cancer Primer Library II |
|||
HCCP-II | Real Time Primers | 1 set | EUR 657.6 |
Human Cytokine and Chemokine Receptor Primer Library |
|||
HCCR-I | Real Time Primers | 1 set | EUR 774 |
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.