Amyotrophic lateral sclerosis is a fatal neurodegenerative disease caused by the loss of motor neurons in the brain and spinal cord. Amyotrophic lateral sclerosis is currently incurable and treatment drugs are limited and can only delay the condition. The latest research shows that α-linolenic acid can prolong the survival period of patients with amyotrophic lateral sclerosis. This article reviews the neuroprotective effects of α-linolenic acid on amyotrophic lateral sclerosis through different mechanisms of action, aiming at providing some references for the treatment of amyotrophic lateral sclerosis.

Objective:To summarize the clinical manifestations and determine the molecular etiology for three KCNMA1-related neurological disorders.Methods:A retrospective clinical data analysis was performed on 3 patients with clinically and genetically diagnosed neurological diseases related to KCNMA1 gene variants who were diagnosed and treated in the Department of Pediatrics, Xiangya Hospital of Central South University from January 2020 to December 2022.Results:Case 1, a 4-year-old and 8-month-old female, was diagnosed with " episodes of head-raising and lowering for 11 months". The electroencephalogram (EEG) showed background theta rhythm in the occipital area, with mainly sharp waves and peaks in the bilateral central, parietal, occipital and temporal areas. Slow waves were scattered or paroxysmal, affecting the central, parietal, and temporal areas on the right side. Levetiracetam was given as an anti-epileptic treatment, and the seizures were completely controlled. Physical examination revealed unclear articulation and short stature. The patient′s mental development has been delayed since childhood. After 2 years of rehabilitation treatment, his motor development was normal and his language development was slightly delayed. Whole-exome sequencing found a novel c. 1807A>G (p.Thr603Ala) mutation in the KCNMA1 gene, which was graded likely pathogenic (LP). Case 2, a male, 1 year and 4 months old, went to the hospital because of " recurrent stupor attacks for more than 6 months". Nearly 20 epileptic events were detected on the electroencephalogram. Levetiracetam, sodium valproate and clonazepam were administered successively. Seizure treatment, complete seizure control. Brain magnetic resonance imaging (MRI) showed poor cerebellar development. Development was lagging behind that of normal children of the same age. Whole-exome sequencing found a novel c. 756C>A (p.Phe252Leu) mutation in the KCNMA1 gene, which was graded LP. Case 3, a 16-month-old female, went to the hospital because of " trembling upper limbs, slurred speech for more than 10 years, and unsteady gait for 8 years." The electroencephalogram showed no abnormalities, and brain MRI showed cerebellar atrophy. Physical examination: unclear speech, unsteady grasping of objects with both hands, muscle strength of limbs level 4, and ataxic gait. Whole-exome sequencing found a novel c. 1051T>C (p.Ser351Pro) mutation in the KCNMA1 gene, which was graded LP.Conclusions:The core phenotypes of KCNMA1 gene mutation-related neurological diseases include epilepsy, neurodevelopmental disorders and paroxysmal dyskinesia, and cerebellar atrophy is common in brain MRI.

Cannabinoid has attracted extensive attention in recent years, and its medicinal potentials have been gradually explored. Cannabinoid has been approved for Dravet syndrome and Lennox Gastaut syndrome by United States Food and Drug Administration. Recent research shows that cannabinoid is also applicable to tuberous sclerosis related epilepsy. This article reviews the history, mechanism, efficacy and safety of cannabinoid in treating tuberous sclerosis related epilepsy, and interactions between cannabinoid and other antiepileptic drugs so as to provide ideas for tuberous sclerosis related epilepsy.

Objective:To dynamically describe the drug concentration change in different time in vivo to understand the in vivo be-havior patterns and rules of drugs, provide reference for performing individual treatment and avoiding adverse drug reactions and lay foundation for dynamic description of physiological pharmacokinetic model. Methods:A two-compartment model was established using Simulink dynamic simulation and used in curve fitting and parameter estimation. The results from the model were compared with those from 3P97 software. Results:There was no significant difference between the results from the dynamic model and those from 3P97 soft-ware. Conclusion:The dynamic model can be used to dynamically simulate two-compartment model for oral administration. The re-sults from the dynamic simulation are more direct, and can correct the fitting error in 3P97 software.

Objective To analyze the epitopes of anti-hepatitis C virus(HCV)antibodies by peptide library biopanning. Methods Phage random peptide library of 12 amino acids was immunoscreened with purified IgG from the sera of hepatitis C patients. Positive clones which were obtained after 3 rounds of biopanning were detected by ELISA and 4 of them were sequenced. Results After 3 rounds of screening, the radio of output to input increased from (4.6×10~(-4))% to (5.3×10~(-2))%, meaning the enrichment was effective. At the third round of screening, all the selected clones proved to specifically react with the sera for immunoscreening. Four positive phage clones were sequenced, which shared a very conservative sequence and was named as C1. Its inserting sequence in the coat protein III was deduced to be GSMSPYVRWYTP, and the positive rate of C1 reacted with 20 cases of HCV patients was 85%.Conclusion The antigen-mimic peptide C1 is successfully screened from 12 random phage peptide library and it has some diagnostic value.

OBJECTIVETo investigate spontaneous metastasis, micrometastasis and genetic stability in human breast carcinoma xenografts in nude mice.

METHODSIntact tissue from surgical specimens from breast carcinoma patients was xenografted into nude mice and transplanted from generation to generation. Cells from the xenografts were cultured in vitro and retransplanted into nude mice. Microsatellite DNA in the genome of human breast carcinomas, xenotransplanted tumors and metastases in nude mice were analyzed at three microsatellite loci.

RESULTSThe tumorigenicity of orthotopic xenotransplantation was 88.6% (31/35), with a metastatic rate of 41.9% (13/31). Cells from xenotransplants were successfully cultured in vitro. The taking rate of retransplantation into nude mice and the spontaneous lung metastasis rate were both 100% (10/10). Microsatellite DNA sequences in the genome of xenotransplanted tumors and metastases in nude mice were identical with that of the original human breast carcinoma at three microsatellite loci.

CONCLUSIONSTumorigenicity and metastatic potential can be improved in human breast carcinoma xenografts using intact fresh tumor tissue and orthotopic grafts. Xenotransplanted tumors and tumors after serial passage maintained the genetic stability. The detection of microsatellite DNA may identify micrometastases in a nude mouse model.

Aneuploidy ; Animals ; Breast Neoplasms ; genetics ; pathology ; Cell Division ; Female ; Humans ; Mammary Neoplasms, Experimental ; genetics ; pathology ; Mice ; Mice, Nude ; Microsatellite Repeats ; Neoplasm Metastasis ; Neoplasm Transplantation ; Time Factors ; Transplantation, Heterologous ; Tumor Cells, Cultured