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Spatial and temporal distribution characteristics of synthetic musk in Suzhou Creek

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Journal of Shanghai University(English Edition) 2010年第4期14卷 306-311页

作者：王凤玲周赢郭亚文邹立寅张晓岚曾祥英 School of Environmental and Chemical Engineering Shanghai University State Key Laboratory of Organic Geochemistry Guangzhou Institute of GeochemistryChinese Academy of SciencesGuangzhou 510640P.R.China

Contamination levels and distribution of seven synthetic musks in Suzhou Creek were investigated in this study. Two polycyclic musks, 1, 3, 4, 6, 7, 8-hexahydro-4, 6, 6, 7, 8, 8-hexamethylcyclopenta（g）-2-benzopyran （HHCB） and 7-acetyl-1, 1, 1, 3, 4, 4, 6-hexamethyl-1, 2, 3, 4-tetrahydronaphthalene （AHTN）, were found in all samples. The ranges of HHCB and AHTN were from 8 ng/L to 138 ng/L and from 1 ng/L to 25 ng/L in surface waters, and from 2 ng/g to 77 ng/g （dry weight） and from 1 ng/g to 31 ng/g （dry weight） in sediments respectively. The total concentration of HHCB and AHTN showed obvious spatial change in surface waters： the upper reaches （suburb） 13 less than the lower course （urban area）; and in sediments, the musk levels were also higher in urban area. The distribution pattern also showed seasonaly varied characteristics both in surface waters and sediments. The distribution coefficient （Kp） and standardized distribution coefficient （Koc） were calculated through musk concentrations and octanol-water partition coefficient （Kow）. The results showed that AHTN was more easily absorbed in sediments. The adsorption of musk was increased with the increasing of total organic carbon （TOC） in Suzhou Creek. The significant positive correlation of HHCB and AHTN was found in this study, which indicated the same pollution source： the use and discharge of flavoring substances.

关键词： musk Suzhou Creek surface waters sediments

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musk抗体阳性重症肌无力10例临床分析

中国神经精神疾病杂志 2020年第1期46卷 24-28页

作者：沈发秀李尊波雷霖范志荣卢岩王敏邸丽徐敏陈海王锁彬笪宇威首都医科大学宣武医院神经内科北京100053 西安高新医院神经内科

目的探讨骨骼肌特异性酪氨酸激酶(muscle-specific tyrosine kinase,musk)抗体阳性重症肌无力(musk-myasthenia gravis,MMG)临床特点和对治疗的反应。方法分析2017年7月至2018年12月就诊于我院的MG患者的临床资料,计算MMG占全部MG患者比... 详细信息

目的探讨骨骼肌特异性酪氨酸激酶(muscle-specific tyrosine kinase,musk)抗体阳性重症肌无力(musk-myasthenia gravis,MMG)临床特点和对治疗的反应。方法分析2017年7月至2018年12月就诊于我院的MG患者的临床资料,计算MMG占全部MG患者比例,并对MMG患者的起病年龄,起病方式,临床进展模式和对治疗的反应进行分析,并随访至少6个月,每3个月电话或面对面随访记录患者日常生活能力评分(myasthenia gravis-activity of daily living score,MG-ADL)。结果401例MG患者中,完成musk检测319例,musk抗体阳性率为3.13%(10/319)。10例MMG患者男女比为1:9,起病年龄17耀50岁,平均31.4岁。90%患者以眼外肌无力为首发症状。1年内进展为全身型的比例高达90%。溴吡斯的明有效率90%,重频刺激低频递减阳性率为77.78%,无胸腺异常者。经过至少6个月的随访,大多数患者ADL评分降至0耀1分。结论眼动障碍和球部肌无力为这组患者突出的临床表现,免疫治疗有效。

关键词：铱重症肌无力 musk 抗体胆碱酯酶抑制剂免疫抑制剂揖

musk抗体阳性重症肌无力患者面肌的重复神经刺激

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世界核心医学期刊文摘(神经病学分册) 2006年第8期 32-33页

作者： Oh S.J. Hatanaka Y. Hemmi S. 李青波 Department of Neurology University of Alabama at Birmingham Birmingham AL United States Dr.

To better define electrophysiological abnormalities in myasthenia gravis (MG) patients with muscle- specific tyrosine kinase (musk) antibodies (Ab), we compared electrophysiological features of 14 musk Ab- positive, 73 acetylcholine receptor antibody (AChR Ab)- positive, and 22 musk and AChR Ab- negative (seronegative) patients with generalized disease. Repetitive nerve stimulation (RNS) abnormalities were observed in 86% of musk Ab- positive and 82% of AChR Ab- positive patients but in only 55% of seronegative patients. RNS decrements in the orbicularis oculi were more common and severe in the musk Ab- positive patients than the other two groups. Single- fiber electromyography (SFEMG) of the extensor digitorum communis was abnormal in 90% of musk Ab- positive patients. The high frequency of RNS abnormalities in facial muscles in the musk Ab- positive population reflects the propensity for facial muscle involvement in this form of MG and emphasizes the importance of including facial muscles in RNS protocols when evaluating these patients.

关键词：重症肌无力神经肌肉疾病 musk 面肌重复神经刺激抗体阳性患者

musk抗体相关重症肌无力时颜面和延髓肌受累的MRI和临床研究

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世界核心医学期刊文摘(神经病学分册) 2006年第11期 11-12页

作者： Farrugia M.E Robson M.D Clover L A. Vincent 郭俊 Neurosciences Group Weatherall Institute of Molecular Medicine John Radcliffe Hospital Oxford OX3 9DS United Kingdom Prof.

A proportion of patients with myasthenia gravis (MG) without acetylcholine receptor (AChR) antibodies have antibodies to muscle-specific kinase (musk). MG with musk antibodies (musk-MG) is often associated with persistent bulbar involvement, including marked facial weakness and tongue muscle wasting. The extent of muscle wasting in musk-MG, and whether it is also found in the few acetylcholine receptor (AChR-MG) patients who have persistent bulbar involvement, is not clear. We studied 12 musk-MG patients and recruited 14 AChR-MG patients matched broadly for age, sex ratio, duration of disease and degree of ocular, bulbar and facial weakness. We used coronal and sagittal T1-weighted (T1W) and T2-weighted (T 2W) magnetic resonance imaging (MRI) to assess muscle wasting in facial and tongue muscles. Hyperintense signal on T1W MRI and comparison of axial T1W sequences with cUTE sequences were used to assess fibrous/fatty tissue in the tongue. We compared the results with those of four patients with myotonic dystrophy and 12 healthy individuals. We correlated the changes with clinical and treatment histories, and established a new ocular-bulbar-facial-respiratory (OBFR) score. At the time of study, none of the clinical measures, including the OBFR score, differed between the two MG groups. MRI demonstrated thinning of the buccinator, orbicularis oris (***) and orbicularis oculi (***) muscles in musk-MG patients compared with healthy controls, whereas thinning of these muscles was not significant in AChR-MG. Tongue areas with T1W high signal were increased in musk-MG patients and the intensity of the signal on axial T1W sequences was greater in musk-MG than in controls. To look for possible correlations between imaging and clinical findings, we pooled results from all MG patients. The duration of treatment with prednisolone at >40 mg on alternate days (AD) correlated positively with the percentage of tongue area with high signal (P = 0.006) and negatively with MRI

关键词：重症肌无力神经肌肉疾病延髓脑干 musk 颜面抗体

WeChat,Elon musk and"the Everything App" Could the West Get Its First Super App?

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城市漫步（上海版、英文） 2022年第11期 23-23页

作者： Lars James Hamer 不详

Elon musk has completed the takeover of the social media platform Twitter for a staggering USD44 billion,reports the *** move was completed on Friday,October 28,2022,bringing an end to the saga that almost saw the two... 详细信息

关键词： bringing completed musk

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仅有眼部症状的抗musk型重症肌无力

世界核心医学期刊文摘（眼科学分册） 2005年第11期1卷 5-5页

作者： Caress J.B. Hunt C.H. Batish S.D. 杨秀梅 Wake Forest University School of Medicine Department of Neurology Medical Center Boulevard Winston-Salem NC 27157 United StatesDr.

Background: Antibodies to a muscle-specific receptor tyrosine kinase (musk) h ave been found in approximately 40%of patients with generalized myasthenia grav is who are seronegative for the antiacetylcholine receptor antibody. Many of the patients with anti-musk antibodies have prominent oculobulbar symptoms or weak ness of the neck and respiratory muscles, but patients with ocular myasthenia ha ve not been described. Objective: To report a case of ocular myasthenia due to a nti-musk antibodies. Patient: A young woman with ocular myasthenia and antibodi es to musk. Results: Anti-musk antibody was detected by radioimmunoassay using highly purified musk recombinant antigen. Conclusion: Ocular myasthenia gravis i s a presentation of the anti-musk antibody syndrome.

关键词：重症肌无力 musk 眼部症状肌无力患者乙酰胆碱受体肌力减弱放射免疫分析法重组抗原呼吸肌无眼

利妥昔单抗成功治疗musk抗体阳性重症肌无力患者

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世界核心医学期刊文摘(神经病学分册) 2006年第8期 34-34页

作者： Hain B. Jordan K. Deschauer M. Zierz S. 李青波 Department of Neurology Martin Luther University of Halle- Wittenberg Ernst- Grube- Strasse 40 06120 Hal- le/SaaleGermany Dr.

We report on a 56- year- old woman with muscle- specific receptor tyrosine kinase (musk) antibody- positive myasthenia with predominant bulbar symptoms and respiratory insufficiency. Conventional immunosuppression (predniso- lone, azathioprine, mycophenolate mofetil) could not maintain the clinical improvement initially achieved by repeated plasma exchanges. Therefore, treatment with rituximab was initiated. After 2 months of rituximab treatment, remarkable clinical improvement correlating with a reduction of musk serum antibodies was seen. The patient continued to remain stable 12 months after initiation of therapy. This case report demonstrates that rituximab may be an effective and tolerable treatment in musk antibody- positive myasthenia gravis.

关键词：重症肌无力单抗神经肌肉疾病利妥昔抗体阳性 musk 患者

Effect of natural extract eye drops in dry eye disease rats

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International Journal of Ophthalmology(English edition) 2020年第7期13卷 1023-1030页

作者： Soo Youn Choi Youngsub Eom Jee Yong Kim Dong Hoon Jang Jong Suk Song Hyo Myung Kim Department of Ophthalmology Korea University College of MedicineSeoul 02841Republic of Korea BGN World Tower Eye Clinic Seoul 05551Republic of Korea Department of Ophthalmology Korea University Ansan HospitalKorea University College of MedicineGyeonggi-do 15355Republic of Korea Medical O Co. Ltd.Seoul 06025Republic of Korea

AIM:To investigate the therapeutic effect of natural extract eye drops containing bee venom,musk,and deer antlers in dry eye disease(DED)animal ***:Scopolamine-injected DED rats and lacrimal gland-excised rats were allocated into control,saline,and natural extract groups respectively and a normal group(lacrimal gland excision was not performed)in lacrimal gland-excised *** eye drop instillation 4 times a day for 5 d,corneal fluorescein staining(CFS)scores,tear MUC5AC levels,and tear lactic dehydrogenase(LDH)levels were ***:In scopolamine-injected rats,the natural extract-treated group had significantly lower CFS scores(1.7±0.5,4.7±1.4,3.8±1.9,P=0.006)and tear LDH levels(0.10±0.01,0.19±0.01,0.16±0.08 OD,P=0.014)but higher tear MUC5AC levels(12.9±3.7,7.9±2.0,9.7±3.6 ng/m L,P=0.041)compared with the control and saline-treated *** were no significant differences between the control and saline-treated *** lacrimal gland-excised rats,the natural extract-treated group also had lower CFS scores(4.3±1.2,11.5±2.3,9.0±1.9,Pmusk,and deer antlers may have effectiveness in DED treatment by restoring the damaged ocular surface,increasing tear volume,and recovering the tear mucin layer in DED rats.

关键词： bee venom musk deer antler natural extract dry eye disease rat

Possible implications of dysregulated nicotinic acetylcholine receptor diffusion and nanocluster formation in myasthenia gravis

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Neural Regeneration Research 2021年第2期16卷 242-246页

作者： Francisco J.Barrantes Biomedical Research Institute(BIOMED)UCA-CONICET Buenos AiresArgentina

Myasthenia gravis is a rare and invalidating disease affecting the neuromuscular junction of voluntary *** classical form of this autoimmune disease is characterized by the presence of antibodies against the most abundant protein in the neuromuscular junction,the nicotinic acetylcholine *** variants of the disease involve autoimmune attack of non-receptor scaffolding proteins or enzymes essential for building or maintaining the integrity of this peripheral *** review summarizes the participation of the above proteins in building the neuromuscular junction and the destruction of this cholinergic synapse by autoimmune aggression in myasthenia *** review also covers the application of a powerful biophysical technique,superresolution optical microscopy,to image the nicotinic receptor in live cells and follow its motional *** hypothesis is entertained that anomalous nanocluster formation by antibody crosslinking may lead to accelerated endocytic internalization and elevated turnover of the receptor,as observed in myasthenia gravis.

关键词： agrin autoimmune diseases muscle end-plate muscle specific kinase musk myasthenia gravis nanoscopy neuromuscular junction nicotinic acetylcholine receptor rapsyn superresolution microscopy