The human brain contains an estimated 100 billion neurons that must be systematically organized into functional neural circuits for it to function properly.These circuits range from short-range local signaling network...The human brain contains an estimated 100 billion neurons that must be systematically organized into functional neural circuits for it to function properly.These circuits range from short-range local signaling networks between neighboring neurons to long-range networks formed between various brain regions.Compelling converging evidence indicates that alterations in neural circuits arising from abnormalities during early neuronal development or neurodegeneration contribute significantly to the etiology of neurological disorders.Supporting this notion,efforts to identify genetic causes of these disorders have uncovered an over-representation of genes encoding proteins involved in the processes of neuronal differentiation,maturation,synaptogenesis and synaptic function.Fasciculation and elongation protein zeta-1,a Kinesin-1 adapter,has emerged as a key central player involved in many of these processes.Fasciculation and elongation protein zeta-1-dependent transport of synaptic cargoes and mitochondria is essential for neuronal development and synapse establishment.Furthermore,it acts downstream of guidance cue pathways to regulate axo-dendritic development.Significantly,perturbing its function causes abnormalities in neuronal development and synapse formation both in the brain as well as the peripheral nervous system.Mutations and deletions of the fasciculation and elongation protein zeta-1 gene are linked to neurodevelopmental disorders.Moreover,altered phosphorylation of the protein contributes to neurodegenerative disorders.Together,these findings strongly implicate the importance of fasciculation and elongation protein zeta-1 in the establishment of neuronal circuits and its maintenance.展开更多
Objective To determine the diagnostic accuracy of the intensity of fasciculation evaluated by muscle ultrasound in the differential diagnosis of amyotrophic lateral sclerosis(ALS).Methods We prospectively recruited pa...Objective To determine the diagnostic accuracy of the intensity of fasciculation evaluated by muscle ultrasound in the differential diagnosis of amyotrophic lateral sclerosis(ALS).Methods We prospectively recruited patients who had ALS and neuropathy-radiculopathy attending Peking Union Medical College Hospital from 2017 to 2020.Healthy adults from a community were recruited as healthy controls.Muscle strength was assessed using the Medical Research Council(MRC)scale.At the first visit to the hospital,patients were assessed for maximal grade of fasciculations,total fasciculation score,and fasciculation grade in 16 muscle groups of bilateral upper and lower limbs using ultrasonography.The sensitivity and specificity of maximal grade of fasciculations,total fasciculation score,and fasciculation grade for the diagnosis of ALS were assessed by receiver operating characteristic analyses.Results The percentage of limb muscles with a maximal fasciculation grade higher than grade 2 in ALS patients and neuropathy-radiculopathy patients was 84.9%and 9.8%,respectively(χ^(2)=172.436,P<0.01).Of the 16 limb muscles detected,the total fasciculation score[median(interquartile range)]was 29(15,41)in ALS patients and 3(0,8)in neuropathy-radiculopathy patients(Z=9.642,P<0.001).Remarkable fasciculations were seen in ALS patients whose muscles with a MRC score ranging from 2 to 4,followed by patients with MRC score 5,and then in those with MRC score 0 and 1.The sensitivity and specificity of total fasciculation score for diagnosis of ALS were 80.6%and 93.4%,respectively(cut-off value 14).In patients with ALS,for muscles with MRC score 4 and 5,the percentage of muscles with fasciculation grades≥3 was 42.3%and 24.1%respectively,while in neuropathy-radiculopathy patients,the percentage for muscles with MRC score 4 and 5 was only 1.7%and 0,respectively.Conclusion A combined analysis of fasciculation intensity and MRC score of the limb muscles may be helpful for differential diagnosis of ALS.展开更多
General anesthesia with controlled ventilation through endotracheal tube is an anaesthetic technique used by the anesthesiologists all over the world. Laryngoscopy and endotracheal intubation is necessary to achieve t...General anesthesia with controlled ventilation through endotracheal tube is an anaesthetic technique used by the anesthesiologists all over the world. Laryngoscopy and endotracheal intubation is necessary to achieve this technique. Traditionally succinylcholine is drug of choice for induction due to its rapid onset of action. Aim: This study compared the efficiency of magnesium sulphate in reducing fasciculation and increase in serum potassium which are the common complications associated with suxamethonium administration. Methods: Fifty six patients being planned for lower abdominal surgery under general anaesthesia were randomized into two groups M and S. Serum potassium was taken in the theatre, three minutes before induction of anesthesia patients in M group were given 6 mg/kg of magnesium sulphate diluted in 10 ml normal saline intravenously while in S group 10 ml of normal saline was injected intravenously. The occurrence and degree of fasciculation were recorded after administration of suxamethonium in both groups while serum potassium was also taken at the end of fasciculations. The researcher was blinded to the content of the syringes until after the study. Results: More patients in the S group had fasciculations compared to the M group while the degree of fasciculations was more in the S group, the serum potassium increase was more in the saline group.展开更多
Benign fasciculation Syndrome (BFS) is related with anxiety level;it is known as fasciculation anxiety syndrome. It may also be caused by long-term use of steroid or anticholinergic, as well as nicotine, caffeine, alc...Benign fasciculation Syndrome (BFS) is related with anxiety level;it is known as fasciculation anxiety syndrome. It may also be caused by long-term use of steroid or anticholinergic, as well as nicotine, caffeine, alcohol, and exposure to insecticides and pesticides. This paper presents a benign fasciculation syndrome case developing after CoronaVac vaccination.展开更多
The formation of nerve bundles,which is partially regulated by neural cell adhesion molecule 1(NCAM1),is important for neural network organization during peripheral nerve regeneration.However,little is known about how...The formation of nerve bundles,which is partially regulated by neural cell adhesion molecule 1(NCAM1),is important for neural network organization during peripheral nerve regeneration.However,little is known about how the extracellular matrix(ECM)microenvironment affects this process.Here,we seeded dorsal root ganglion tissue blocks on different ECM substrates of peripheral nerve ECM-derived matrixgel,Matrigel,laminin 521,collagen I,and collagen IV,and observed well-aligned axon bundles growing in the peripheral nerve ECM-derived environment.We confirmed that NCAM1 is necessary but not sufficient to trigger this phenomenon.A protein interaction assay identified collagen VI as an extracellular partner of NCAM1 in the regulation of axonal fasciculation.Collagen VI interacted with NCAM1 by directly binding to the FNIII domain,thereby increasing the stability of NCAM1 at the axolemma.Our in vivo experiments on a rat sciatic nerve defect model also demonstrated orderly nerve bundle regeneration with improved projection accuracy and functional recovery after treatment with 10 mg/m L Matrigel and 20μg/m L collagen VI.These findings suggest that the collagen VI-NCAM1 pathway plays a regulatory role in nerve bundle formation.This study was approved by the Animal Ethics Committee of Guangzhou Medical University(approval No.GY2019048)on April 30,2019.展开更多
zpl (zip-like) gene mutant embryos showed the cuticular defect with alternative denticle rows and a hole from head to abdomen. zpl mutants also caused the overgrowth of neural cells and axons both in CNS and PNS as we...zpl (zip-like) gene mutant embryos showed the cuticular defect with alternative denticle rows and a hole from head to abdomen. zpl mutants also caused the overgrowth of neural cells and axons both in CNS and PNS as well as the wrong pathway of neural fasciculation and the disappearance of hypophysis, as shown by whole mount embryos stained withantibody against HRP and MAb-22C10. Genetic analysis has protided evidence that zpl, located in the right arm ofthe second cliromosome (between 75 and 102 genetic map units), is a new gene closely related to the zip gene.展开更多
基金the Singapore Ministry of Education(T1-2015 Apr-03)the National University of SingaporeInstitute for Health Innovation and Technology to JJEC.
文摘The human brain contains an estimated 100 billion neurons that must be systematically organized into functional neural circuits for it to function properly.These circuits range from short-range local signaling networks between neighboring neurons to long-range networks formed between various brain regions.Compelling converging evidence indicates that alterations in neural circuits arising from abnormalities during early neuronal development or neurodegeneration contribute significantly to the etiology of neurological disorders.Supporting this notion,efforts to identify genetic causes of these disorders have uncovered an over-representation of genes encoding proteins involved in the processes of neuronal differentiation,maturation,synaptogenesis and synaptic function.Fasciculation and elongation protein zeta-1,a Kinesin-1 adapter,has emerged as a key central player involved in many of these processes.Fasciculation and elongation protein zeta-1-dependent transport of synaptic cargoes and mitochondria is essential for neuronal development and synapse establishment.Furthermore,it acts downstream of guidance cue pathways to regulate axo-dendritic development.Significantly,perturbing its function causes abnormalities in neuronal development and synapse formation both in the brain as well as the peripheral nervous system.Mutations and deletions of the fasciculation and elongation protein zeta-1 gene are linked to neurodevelopmental disorders.Moreover,altered phosphorylation of the protein contributes to neurodegenerative disorders.Together,these findings strongly implicate the importance of fasciculation and elongation protein zeta-1 in the establishment of neuronal circuits and its maintenance.
基金funded by the CAMS Innovation Fund for Medical Sciences(CIFMS 2021-I2M-1-003)National High Level Hospital Clinical Research Funding(2022-PUMCH-B-017)The Strategic Priority Research Program(Pilot study)"Biological Basis of Aging and Therapeutic Strategies"of the Chinese Academy of Sciences(grant XDB39040000).
文摘Objective To determine the diagnostic accuracy of the intensity of fasciculation evaluated by muscle ultrasound in the differential diagnosis of amyotrophic lateral sclerosis(ALS).Methods We prospectively recruited patients who had ALS and neuropathy-radiculopathy attending Peking Union Medical College Hospital from 2017 to 2020.Healthy adults from a community were recruited as healthy controls.Muscle strength was assessed using the Medical Research Council(MRC)scale.At the first visit to the hospital,patients were assessed for maximal grade of fasciculations,total fasciculation score,and fasciculation grade in 16 muscle groups of bilateral upper and lower limbs using ultrasonography.The sensitivity and specificity of maximal grade of fasciculations,total fasciculation score,and fasciculation grade for the diagnosis of ALS were assessed by receiver operating characteristic analyses.Results The percentage of limb muscles with a maximal fasciculation grade higher than grade 2 in ALS patients and neuropathy-radiculopathy patients was 84.9%and 9.8%,respectively(χ^(2)=172.436,P<0.01).Of the 16 limb muscles detected,the total fasciculation score[median(interquartile range)]was 29(15,41)in ALS patients and 3(0,8)in neuropathy-radiculopathy patients(Z=9.642,P<0.001).Remarkable fasciculations were seen in ALS patients whose muscles with a MRC score ranging from 2 to 4,followed by patients with MRC score 5,and then in those with MRC score 0 and 1.The sensitivity and specificity of total fasciculation score for diagnosis of ALS were 80.6%and 93.4%,respectively(cut-off value 14).In patients with ALS,for muscles with MRC score 4 and 5,the percentage of muscles with fasciculation grades≥3 was 42.3%and 24.1%respectively,while in neuropathy-radiculopathy patients,the percentage for muscles with MRC score 4 and 5 was only 1.7%and 0,respectively.Conclusion A combined analysis of fasciculation intensity and MRC score of the limb muscles may be helpful for differential diagnosis of ALS.
文摘General anesthesia with controlled ventilation through endotracheal tube is an anaesthetic technique used by the anesthesiologists all over the world. Laryngoscopy and endotracheal intubation is necessary to achieve this technique. Traditionally succinylcholine is drug of choice for induction due to its rapid onset of action. Aim: This study compared the efficiency of magnesium sulphate in reducing fasciculation and increase in serum potassium which are the common complications associated with suxamethonium administration. Methods: Fifty six patients being planned for lower abdominal surgery under general anaesthesia were randomized into two groups M and S. Serum potassium was taken in the theatre, three minutes before induction of anesthesia patients in M group were given 6 mg/kg of magnesium sulphate diluted in 10 ml normal saline intravenously while in S group 10 ml of normal saline was injected intravenously. The occurrence and degree of fasciculation were recorded after administration of suxamethonium in both groups while serum potassium was also taken at the end of fasciculations. The researcher was blinded to the content of the syringes until after the study. Results: More patients in the S group had fasciculations compared to the M group while the degree of fasciculations was more in the S group, the serum potassium increase was more in the saline group.
文摘Benign fasciculation Syndrome (BFS) is related with anxiety level;it is known as fasciculation anxiety syndrome. It may also be caused by long-term use of steroid or anticholinergic, as well as nicotine, caffeine, alcohol, and exposure to insecticides and pesticides. This paper presents a benign fasciculation syndrome case developing after CoronaVac vaccination.
基金supported by the National Natural Science Foundation of China,No.31800892(to JLZ)the Natural Science Foundation of Guangdong Province of China,No.2018A030310254(to YY)a grant from Guangzhou Medical University Start-up Project of China,No.B195002002048(to JLZ)。
文摘The formation of nerve bundles,which is partially regulated by neural cell adhesion molecule 1(NCAM1),is important for neural network organization during peripheral nerve regeneration.However,little is known about how the extracellular matrix(ECM)microenvironment affects this process.Here,we seeded dorsal root ganglion tissue blocks on different ECM substrates of peripheral nerve ECM-derived matrixgel,Matrigel,laminin 521,collagen I,and collagen IV,and observed well-aligned axon bundles growing in the peripheral nerve ECM-derived environment.We confirmed that NCAM1 is necessary but not sufficient to trigger this phenomenon.A protein interaction assay identified collagen VI as an extracellular partner of NCAM1 in the regulation of axonal fasciculation.Collagen VI interacted with NCAM1 by directly binding to the FNIII domain,thereby increasing the stability of NCAM1 at the axolemma.Our in vivo experiments on a rat sciatic nerve defect model also demonstrated orderly nerve bundle regeneration with improved projection accuracy and functional recovery after treatment with 10 mg/m L Matrigel and 20μg/m L collagen VI.These findings suggest that the collagen VI-NCAM1 pathway plays a regulatory role in nerve bundle formation.This study was approved by the Animal Ethics Committee of Guangzhou Medical University(approval No.GY2019048)on April 30,2019.
文摘zpl (zip-like) gene mutant embryos showed the cuticular defect with alternative denticle rows and a hole from head to abdomen. zpl mutants also caused the overgrowth of neural cells and axons both in CNS and PNS as well as the wrong pathway of neural fasciculation and the disappearance of hypophysis, as shown by whole mount embryos stained withantibody against HRP and MAb-22C10. Genetic analysis has protided evidence that zpl, located in the right arm ofthe second cliromosome (between 75 and 102 genetic map units), is a new gene closely related to the zip gene.
