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Mitochondrial neuronal uncoupling proteins:a target for potential disease-modification in Parkinson’s disease Philip

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Translational Neurodegeneration 2012年第1期1卷 11-19页

作者： WL ho Jessica WM ho Hui-Fang Liu Danny HF So Zero HM Tse koon-ho chan David B Ramsden Shu-Leong ho Division of Neurology Department of MedicineUniversity of Hong KongPokfulamHong Kong SARChina School of Medicine and School of Biosciences University of BirminghamBirminghamUK Research Centre of Heart BrainHormone and Healthy Aging(HBHA)University of Hong KongPokfulamHong Kong SARChina

This review gives a brief insight into the role of mitochondrial dysfunction and oxidative stress in the converging pathogenic processes involved in Parkinson’s disease(PD).Mitochondria provide cellular energy in the form of ATP via oxidative phosphorylation,but as an integral part of this process,superoxides and other reactive oxygen species are also *** free radical production contributes to oxidative *** have evolved to handle such stress via various endogenous anti-oxidant *** such family of proteins is the mitochondrial uncoupling proteins(UCPs),which are anion carriers located in the mitochondrial inner *** are five known homologues(UCP1 to 5),of which UCP4 and 5 are predominantly expressed in neural *** a series of previous publications,we have shown how these neuronal UCPs respond to 1-methyl-4-phenylpyridinium(MPP+;toxic metabolite of MPTP)and dopamine-induced toxicity to alleviate neuronal cell death by preserving ATP levels and mitochondrial membrane potential,and reducing oxidative *** also showed how their expression can be influenced by nuclear factor kappa-B(NF-B)signaling pathway specifically in ***,we previously reported an interesting link between PD and metabolic processes through the protective effects of leptin(hormone produced by adipocytes)acting via UCP2 against MPP+-induced *** is increasing evidence that these endogenous neuronal UCPs can play a vital role to protect neurons against various pathogenic stresses including those associated with *** expression,which can be induced,may well be a potential therapeutic target for various drugs to alleviate the harmful effects of pathogenic processes in PD and hence modify the progression of this disease.

关键词： uncoupling proteins mitochondria Parkinson??s disease ATP oxidative stress neuroprotection

Variable outcome in infantile-onset inflammatory bowel disease in an Asian cohort

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World Journal of Gastroenterology 2016年第48期22卷 10653-10662页

作者： Way Seah Lee Ruey Terng Ng koon-Wing chan Yu-Lung Lau Department of Paediatrics University Malaya Medical Center University Malaya Paediatric and Child Health Research Group University Malaya Department of Paediatrics and Adolescent Medicine Queen Mary Hospital LKS Faculty of Medicine The University of Hong Kong

AIM Infantile-onset inflammatory bowel disease(IO-IBD) with the onset of disease before 12 mo of age, is a different disease entity from childhood IBD. We aimed to describe the clinical features, outcome and role of mutation in interleukin-10(IL-10) and interleukin-10 receptors(IL-10R) in Asian children with IO-IBD. METhoDS All cases of IO-IBD, defined as onset of disease before 12 mo of age, seen at University Malaya Medical Center, Malaysia were reviewed. We performed mutational analysis for IL10 and IL10 R genes in patients with presenting clinical features of Crohn's disease(CD).RESULTS Six [13%; CD = 3, ulcerative colitis(UC) = 2, IBDunclassified(IBD-U) = 1] of the 48 children(CD = 25; UC = 23) with IBD have IO-IBD. At final review [median(range) duration of follow-up: 6.5(3.0-20) years], three patients were in remission without immunosuppression [one each for post-colostomy(IBD-U), after standard immunosuppression(CD), and after total colectomy(UC)]. Three patients were on immunosuppression:one(UC) was in remission while two(both CD) had persistent disease. As compared with later-onset disease, IO-IBD were more likely to present with bloody diarrhea(100% vs 55%, P = 0.039) but were similar in terms of an associated autoimmune liver disease(0% vs 19%, P = 0.31), requiring biologics therapy(50% vs 36%, P = 0.40), surgery(50% vs 29%, P = 0.27), or achieving remission(50% vs 64%, P = 0.40). No mutations in either IL10 or IL10 R in the three patients with CD and the only patient with IBD-U were *** The clinical features of IO-IBD in this Asian cohort of children who were negative for IL-10 or IL-10 R mutations were variable. As compared to childhood IBD with onset of disease after 12 mo of age, IO-IBD achieved remission at a similar rate.

关键词： Infantile-onset inflammatory bowel disease Pediatric

A converged ubiquitin-proteasome pathway for the degradation of TOC and TOM tail-anchored receptors

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Journal of Integrative Plant Biology 2024年第5期66卷 1007-1023页

作者： Meijing Yang Shuai Chen Shey-Li Lim Lang Yang Jia Yi Zhong koon Chuen chan Zhizhu Zhao Kam-Bo Wong Junqi Wang Boon Leong Lim School of Biological Sciences University of Hong KongPokfulam 999077Hong KongChina Department of Biology Southern University of Science and TechnologyShenzhen 518055China School of Biomedical Engineering Shenzhen UniversityShenzhen 518060China School of Life Sciences The Chinese Universityof Hong KongShatin 999077HongKongChina State Key Laboratory of Agrobiotechnology The Chinese University of Hong KongShatin 999077Hong KongChina HKU Shenzhen Institute of Research and Innovation Shenzhen 518052China

In plants,thousands of nucleus-encoded proteins translated in the cytosol are sorted to chloroplasts and mitochondria by binding to specific receptors of the TOC(translocon on the outer chloroplast membrane)and the TOM(translocon on the outer mitochondrial membrane)complexes for import into those *** degradation pathways for these receptors are ***,we discovered a converged ubiquitin-proteasome pathway for the degradation of Arabidopsis thaliana TOC and TOM tail-anchored *** receptors are ubiquitinated by E3 ligase(s)and pulled from the outer membranes by the AAA+adenosine triphosphatase CDC48,after which a previously uncharacterized cytosolic protein,transmembrane domain(TMD)-binding protein for tail-anchored outer membrane proteins(TTOP),binds to the exposed TMDs at the C termini of the receptors and CDC48,and delivers these complexes to the 26S proteasome.

关键词： 26S proteasome CDC48 SP1 TOC TOM ubiquitination

Prenatal diagnosis of choledochal cyst using magnetic resonance imaging: A case report

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World Journal of Gastroenterology 2005年第32期11卷 5082-5083页

作者： Alex Mun-Ching Wong Yun-Chung Cheung Yu-Hung Liu koon-Kwan Ng Siu-Cheung chan Shu-Hang Ng Department of Diagnostic Radiologychang Gung Memorial hospital Kwei-ShanTao-YuanTaiwanChina Obstetrics and Gynecologychang Gung Memorial hospital 222Mei Chin RoadKeelungTaiwanChina

Choledochal cysts are congenital anomalies of the biliary ducts, characterized by cystic dilatation of the *** diagnosis of this anomaly using ultrasonography (US) has been well documented. Magnetic resonance imaging (MRI) has recently become an important complement to US in prenatal diagnosis of fetal anomalies. We herein report a patient in whom at 24 wk' gestation US suggested a right upper quadrant abdominal cyst and in whom at 26 wk' gestation MRI more clearly delineated the cyst and its surrounding structures and suggested a choledochal cyst, which was confirmed at postnatal surgery and histopathology.

关键词： Choledochal cyst Prenatal diagnosis MRI

Computed tomography findings in fatal cases of enormous hepatic portal venous gas

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World Journal of Gastroenterology 2005年第19期11卷 2953-2955页

作者： Siu-Cheung chan Yung-Liang Wan Yun-Chung Cheung Shu-Hang Ng Alex Mun-Ching Wong koon-Kwan Ng Department of Diagnostic Radiology chang Gung Memorial hospital Linko Medical Center and Keelung HospitalTaoyuanTaiwanChina

AIM: To assess the computed tomography (CT) findings in the patients with hepatic portal venous gas (HPVG) who presented with a short fatal clinical course in our hospital in order to demonstrate if there was any sign for ***: Between January 1997 and December 2000, CT scan of the abdomen was performed on 949 patients with acute abdominal pain in our emergency *** patients were found having HPVG. The CT images and clinical presentations of all these five patients were reviewed. RESULTS: In reviewing the CT findings of the cases, HPVG in bilateral hepatic lobes, abnormal gas in the superior mesenteric veins, small bowel intramural gas, and bowel distension were observed in all patients. Dry gas in multiple branches of the mesenteric vein was also revealed in all cases. All the patients expired due to irreversible septic shock within 48 h after their initial clinical presentation in emergency room. Two patients had acute pancreatitis with grade D and E Balthazar classification and they expired within 24 h due to progressing septic shock under aggressive medical treatment and life support. Two patients with underlying end stage renal disease expired within 48 h even though emergent surgical intervention was undertaken. The excited bowels revealed severe ischemic change. One patient expired only a few hours after the CT examination. CONCLUSION: HPVG is a diagnostic clue in patients with acute abdominal conditions, and CT is the most specific diagnostic tool for its evaluation. The dry mesenteric veins are the suggestive fatal sign, especially for the deteriorating patients, with the direct effect on gastrointestinal perfusion.

关键词： Hepatic portal vein Intestines Ischemia Computed tomography

伴有特殊表型的Wiskott-Aldrich综合征3例

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中国当代儿科杂志 2017年第2期19卷 250-253页

作者：贺建新 chan koon Wing 徐保平刘秀云桂晋刚申昆玲江载芳 Lau Yu Lung 首都医科大学附属北京儿童医院呼吸科北京100045 香港大学儿童与青少年科学系首都医科大学附属北京儿童医院免疫研究室北京100045

病例1 患儿,男,4个月。因发现血小板减少4月余,咳嗽50 d,发热4 d入院。患儿4个月前（生后2 d）发现血小板减少（PLT 22×10^9/L）,外院经骨髓细胞学检查诊断为血小板减少性紫癜,口服泼尼松及静脉丙种球蛋白治疗,血小板正常后出院。出院... 详细信息

病例1 患儿,男,4个月。因发现血小板减少4月余,咳嗽50 d,发热4 d入院。患儿4个月前（生后2 d）发现血小板减少（PLT 22×10^9/L）,外院经骨髓细胞学检查诊断为血小板减少性紫癜,口服泼尼松及静脉丙种球蛋白治疗,血小板正常后出院。出院后不规律口服泼尼松,2个月前停用,血小板波动于50～60×10^9/L。

关键词： Wiskott-Aldrich综合征血小板减少性紫癜静脉丙种球蛋白表型骨髓细胞学检查诊断泼尼松出院后

同一家族内IL-12β1受体缺陷引起的卡介苗播散性感染二例

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同一家族内IL-12β1受体缺陷引起的卡介苗播散性感染二例

Important role of cardiac spect imaging in patients undergoing percutaneous transluminal coronary angioplasty

中华医学会第十四次全国儿科学术会议

作者：王晓川章迁王晓红王莹吕志宝刘宇隆 chan koon Wing 复旦大学附属儿科医院香港大学医学院

目的:报道同一家族中两例IL-12Rβ1受体缺陷引起的卡介苗播散性感染。方法:对第1例患儿进行的是回顾性诊断,对第2例患儿结合其家族史、临床表现和基因诊断进行诊断。结果:例1患儿男性,2000年9月27日出生,生后2小时接种卡介苗,4月龄时发...

目的:报道同一家族中两例IL-12Rβ1受体缺陷引起的卡介苗播散性感染。方法:对第1例患儿进行的是回顾性诊断,对第2例患儿结合其家族史、临床表现和基因诊断进行诊断。结果:例1患儿男性,2000年9月27日出生,生后2小时接种卡介苗,4月龄时发现其左腋下淋巴结结核,给予异烟肼+ 利福平+丁胺卡娜霉素治疗,于2001年4月17日行左腋下淋巴结团块切除术,同年8月行右侧腹股沟淋巴结清扫术,术后继续抗痨治疗。于2001年11月起,患儿出现发热、白蛋白降低并有腹水,继续给

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Chinese Medical Journal 1999年第3期112卷 64-67页

作者： Babington Chun kuen Yung, Wai Kwong chan, Chiu Sun Yue, Wai Chau Fan, Kwok Fai Leung Amy Siu ling Fung and James Chi sang chan Department of Radiology and Organ Imaging (Yung CK, Fan WC, chan CS) & Department of Medicine and Geriatrics ( Department of Radiology and Organ Imaging United Christian Hospital 130 Hip Wo Street Kwun Tong Kowloon Hong Kong China Department of Medicine and Geriatrics United Christian Hospital 130 Hip Wo Street Kwun Tong Kowloon Hong Kong China Department of Radiology Tuen Mun Hospital Tsing Chung Koon Road Tuen Mun Hong Kong China

Objective To examine the utilization and contribution of cardiac perfusion scans to patients undergoing percutaneous transluminal coronary angioplasty. Methods From July 1996 to January 1998, 34 patients underwent percutaneous transluminal coronary angioplasty and cardiac SPECT imaging was studied. Twenty five patients had pre procedure SPECT scans. The agreement between angioplasty strategies and SPECT findings of ischemia and hypokinesia was *** When radionuclide imaging was performed before revascularization procedures, 84% of the patients had intervention strategies guided by scan findings. Perfusion scan findings including extent and severity of ischemia, degree of hypokinesia directed the selection of target vessel for angioplasty in the patient subgroup with multi vessel disease. Post angioplasty radionuclide cardiac scans were performed only on symptomatic patients, demonstrating ischemia and hypokinesia in most of them, conceivably reflected the selection *** We recommend routine pre and post angioplasty radionuclide imaging with multigated scans for wall motion assessment for all patients receiving coronary intervention.

关键词： multigated SPECT angioplasty ischemia hypokinesia

两种新型Wiskott-Aldrich综合征蛋白基因突变的鉴定

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中华儿科杂志 2003年第8期41卷 590-593,T002页

作者：蒋利萍徐酉华杨锡强刘恩梅王莉佳刘宇隆陈冠荣重庆医科大学儿童医院临床免疫室 400014 香港大学医学院儿科系

目的　明确 3例Wiskott Aldrich综合征 (WAS)患儿WAS蛋白 (WASP)基因突变的类型。方法　根据典型临床表现 (血小板减少、湿疹、反复感染 ) ,及淋巴细胞和血小板扫描电镜改变 ,采用PCR直接测序法 ,对 3例疑为WAS的患儿及其母亲的WASP基... 详细信息

目的　明确 3例Wiskott Aldrich综合征 (WAS)患儿WAS蛋白 (WASP)基因突变的类型。方法　根据典型临床表现 (血小板减少、湿疹、反复感染 ) ,及淋巴细胞和血小板扫描电镜改变 ,采用PCR直接测序法 ,对 3例疑为WAS的患儿及其母亲的WASP基因进行序列分析。结果　以正义、反义引物扩增的PCR产物分别测序 ,发现两种新型WASP基因突变 :2例WAS孪生兄弟WASP基因第 10外显子 ,第 984位核苷酸C缺失突变 (984delC) ,导致 317位密码子后移码突变 ,于 4 4 4位密码子提前出现终止密码 (H317fsX4 4 4 ) ;其母亲为此突变WASP基因携带者。另 1例WAS患儿WASP基因第 11外显子 ,第 1388位核苷酸由G替换为T(1388G→T) ,为无义突变 ,使第 4 5 2位密码子提前变为终止密码(E4 5 2X)。其母亲无此突变WASP基因。结论　鉴定出两种新型WASP基因突变 ,WASP基因序列分析对于不典型和散发WAS的诊断及WASP突变基因携带者的检出有重要作用。

关键词： Wiskott-Aldrich综合征基因突变鉴定 WAS蛋白 X-连锁隐性遗传免疫缺陷病小儿