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PHOX2B mutations in three Chinese patients with congenital central hypoventilation syndrome

Chinese Medical Journal 2006年第20期119卷 1749-1752页

作者： Siu-Fong June Or Ming-for Tony Tong Fai-Man Ivan Lo Chi-Wai Law ting-yat miu Delphine Trochet Tak-Sum Stephen Lam Clinical Genetic Service Department of Health Hong Kong SAR China Department of Paediatrics Queen Elizabeth Hospital Hong Kong SAR China Department de Genetique Hospital Necker-Enfants Malades Paris France

Congenital central hypoventilation syndrome （CCHS, OMIM #209880） is a rare autosomal dominant disorder of the autonomic nervous system （ANS） characterized by an abnormal autonomic ventilatory response to progressive hypercarbia and sustained hypoxemia. Patients typically present in the newborn period with hypoventilation or apnea asleep, awake, or both, without any associated cardiac, pulmonary, neuromuscular or brainstem lesions. Rarely, some patients may present at a later age and are diagnosed to have late onset central hypoventilation syndrome （LOCHS）.1 Other features of ANS dysfunction such as feeding difficulty due to oesophageal dysmotility, severe constipation in the absence of Hirschsprung disease, poor regulation of basal body temperature, episodes of profuse sweating, pupillary and ocular abnormalities, decreased beat-to-beat variability of heart rate, attenuated response of heart rate to exercise, abnormal fluctuation of blood pressure, decreased perception to pain, and decreased perception to anxiety may be variably present but not essential for diagnosis Furthermore, this central hypoventilation can occur as an isolated feature or in association with a number of neurocristopathies, notably Hirschsprung disease （Haddad syndrome, OMIM #209880） and tumours of the sympathetic nervous system particularly neuroblastoma, ganglioneuro- blastoma, and ganglioneuroma, which were found in 20% and 5%--10% of CCHS patients, respectively.2 Studies of genes pertinent to the early embryologic development of the neural crest cells, specifically the endothelin and the RET-GDNF signaling pathways, have recently led to the identification of PHOX2B as the major disease causing gene for CCHS.2-6 PHOX2B was mapped to chromosome 4p12 and consists of 3 exons. It encodes a highly conserved paired-like homeobox transcription factor of 314 amino acids linked to the RET-GDNF signaling

关键词： congenital central hypoventilation syndrome PHOX2B Chinese mutation

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Multi-attribute decision making method for air target threat evaluation based on intuitionistic fuzzy sets

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Journal of Systems Engineering and Electronics 2012年第6期23卷 891-897页

作者： Yongjie XU YongchunWang Xudong miu Scientific Research Department Dalian Naval Academy Dalian 116018 R R. China

The function of the air target threat evaluation （TE） is the foundation for weapons allocation and senor resources management within the surface air defense. The multi-attribute evaluation methodology is utilized to address the issue of the TE in which the tactic features of the detected target are treated as evaluation attributes. Meanwhile, the intuitionistic fuzzy set （IFS） is employed to deal with information uncertainty in the TE process. Furthermore, on the basis of the entropy weight and inclusion-comparison probability, a hybrid TE method is developed. In order to accommodate the demands of naturalistic decision making, the proposed method allows air defense commanders to express their intuitive opinions besides incorporating into the threat features of the detected target. An illustrative example is provided to indicate the feasibility and advantage of the proposed method.

关键词： threat evaluation (TE) intuitionistic fuzzy set (IFS),multi-attribute evaluation intuitive opinions entropy weight,inclusion-comparison probability (ICP).

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Adaptive dynamic range shift (ADRIFT) quantitative phase imaging

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Light(Science & Applications) 2021年第1期10卷 19-28页

作者： Keiichiro Toda miu Tamamitsu Takuro Ideguchi Department of Physics The University of TokyoTokyo 113-0033Japan Institute for Photon Science and Technology The University of TokyoTokyo 113-0033Japan PRESTO Japan Science and Technology AgencySaitama 332-0012Japan

Quantitative phase imaging(QPI)with its high-contrast images of optical phase delay(OPD)maps is often used for label-free single-cell *** to other imaging methods,sensitivity improvement has not been intensively explored because conventional QPI is sensitive enough to observe the surface roughness of a substrate that restricts the minimum measurable ***,emerging QPI techniques that utilize,for example,differential image analysis of consecutive temporal frames,such as mid-infrared photothermal QPI,mitigate the minimum OPD limit by decoupling the static OPD contribution and allow measurement of much smaller ***,we propose and demonstrate supersensitive QPI with an expanded dynamic *** is enabled by adaptive dynamic range shift through a combination of wavefront shaping and dark-field QPI *** a proof-of-concept demonstration,we show dynamic range expansion(sensitivity improvement)of QPI by a factor of 6.6 and its utility in improving the sensitivity of mid-infrared photothermal *** technique can also be applied for wide-field scattering imaging of dynamically changing nanoscale objects inside and outside a biological cell without losing global cellular morphological image information.

关键词： scattering dynamic phase

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Determination of primary aromatic amines using immobilized nanoparticles based surface-enhanced Raman spectroscopy

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Chinese Chemical Letters 2016年第5期27卷 745-748页

作者： ting Wu Hai-ting Wang Bo Shen Yi-Ping Du Xuan Wang Zhen-Ping Wang Chuan-Jing Zhang Wen-Bin miu Shanghai Key Laboratory of Functional Materials Chemistry and Research Centre of Analysis and TestEast China University of Science and TechnologyShanghai 200237China Technical Center for Industrial Products and Raw Materials Inspection and Testing Shanghai Entry-Exit Inspection&Inspection and Quarantine Bureau Shanghai 200135China

Primary aromatic amines （PAAs） are substances with toxicity and suspected human carcinogenicity. A facile method for highly sensitive detection of PAAs using surface-enhanced Raman spectroscopy （SERS） is reported. The immobilization of Au nanoparticles （AuNPs） on the glycidyl methacrylate-ethylene dimethacrylate （GMA-EDMA） materials makes the suhstrate a closely packed but not aggregated Au NP arrays which provides a prominent SERS enhancement. Four PAAs with different substituent groups, namely, p-toluidine, p-nitroaniline, benzidine and 4,4-methylene-bis-（2-chloroaniline） have been successfully identified and quantified. High sensitivity and good linear relationship between SERS signals and concentrations of PAAs are obtained for all four PAAs.

关键词： Aromatic amines Surface-enhanced Raman spectroscopy(SERS) Immobilization Au nanoparticles

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Sarcopenia in pancreatic cancer–effects on surgical outcomes and chemotherapy

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World Journal of Gastrointestinal Oncology 2019年第7期11卷 527-537页

作者： miu Yee Chan Kenneth Siu Ho Chok Department of Surgery Queen Mary Hospital 102 Pokfulam Road Hong Kong China Department of Surgery and State Key Laboratory for Liver Research The University of Hong Kong Hong Kong China

Sarcopenia is found in up to 65% of pancreatic cancer patients. The definition and diagnostic methods for sarcopenia have changed over the years, and the measurement of skeletal muscle mass with cross-sectional imaging has become the most popular way of assessment, although the parameters measured vary among different studies. It is still debatable that there is an association between sarcopenia and postoperative pancreatic fistula, but most studies showed a higher risk in patients with sarcopenic obesity. Long-term survival is worse in sarcopenic patients, as shown by meta-analysis. Sarcopenia is also associated with decreased survival and higher toxicity in patients receiving chemotherapy, and chemotherapy also tends to potentiate sarcopenia. Treatment for sarcopenia still remains an area for research, although oral supplements, nutritional modifications and exercise training have been shown to improve sarcopenia.

关键词： Sarcopenia Pancreatic cancer Clinical outcomes Surgical outcomes Chemotherapy Radiotherapy

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Label-free mid-infrared photothermal live-cell imaging beyond video rate

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Light(Science & Applications) 2023年第8期12卷 1661-1674页

作者： Genki Ishigane Keiichiro Toda miu Tamamitsu Hiroyuki Shimada Venkata Ramaiah Badarla Takuro Ideguchi Department of Physics The University of TokyoTokyoJapan Institute for Photon Science and Technology The University of TokyoTokyoJapan

Advancement in mid-infrared(MIR)technology has led to promising biomedical applications of MIR spectroscopy,such as liquid biopsy or breath *** the contrary,MIR microscopy has been rarely used for live biological samples in an aqueous environment due to the lack of spatial resolution and the large water absorption ***,mid-infrared photothermal(MIP)imaging has proven to be applicable to 2D and 3D single-cell imaging with high spatial resolution inherited from visible ***,the maximum measurement rate has been limited to several frames s-1,limiting its range of ***,we develop a significantly improved wide-field MIP quantitative phase microscope with two orders-of-magnitude higher signal-to-noise ratio than previous MIP imaging techniques and demonstrate live-cell imaging beyond video *** first derive optimal system design by numerically simulating thermal conduction following the photothermal ***,we develop the designed system with a homemade nanosecond MIR optical parametric oscillator and a high full-well-capacity image *** high-speed and high-spatial-resolution MIR microscope has great potential to become a new tool for life science,in particular for live-cell analysis.

关键词： beyond conduction limiting

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Purchasing Behaviour of University Students towards Cyber Shopping in Hong Kong

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Journal of Donghua University(English Edition) 2001年第1期18卷 104-108页

作者： Cheng miu-tsz(郑妙紫) Yick Kit-lun(易洁伦) Institute of Textiles and Clothing The Hong Kong Polytechnic University Institute of Textiles and Clothing The Hong Kong Polytechnic University

The developments of Internet and e-commerce provide a new market place to businessman to sell their products and allow people to buy products via Internet as well as to create a new shopping media to consumers. The study aims to investigate the factors affecting consumers' purchase decision via Internet and how product characteristics affect cyber shopping in Hong Kong. The study has revealed that the security of personal data,delivery time, product brand and price were the major concerns for developing cyber market. For marketing apparel products, brand loyalty becomes very important since consumers' judging confidence on the products can be increased in terms of fitting standards and quality aspects.

关键词： cyber shopping, Internet, purchasing behaviour,Hong Kong.

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Mutation of autophagy-related gene ATG7 increases the risk of severe disease in patients with non-alcoholic fatty liver disease

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Liver Research 2023年第4期7卷 365-366页

作者： Mary miu Yee Waye The Nethersole School of Nursing Croucher Laboratory for Human GenomicsThe Chinese University of Hong KongHong KongChina

Non-alcoholic fatty liver disease(NAFLD)is the most common chronic liver disease *** mechanism of NAFLD has been well studied and it has been previously reported that there are some predisposing genetic polymorphisms of NAFLD,e.g.,genes such as fat mass and obesity-associated(FTO)overexpression,leading to triglyceride accumulation,via upregulation of lipogenic genes has been reported.1 An earlier study of a 20-gene set published in 2014 has been correlated with NAFLD severity.2 Adiposity plays an important role in the amplification of the genetic risk of NAFLD-including three variants:PNPLA3,TM6SF2,and GCKR genes.

关键词： NAFLD liver patients

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Outlook of PINK1/Parkin signaling in molecular etiology of Parkinson's disease,with insights into Pink1 knockout models

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Zoological Research 2023年第3期44卷 559-576页

作者： Zhangting Wang See-Wing Chan Hui Zhao Kai-Kei miu Wai-Yee Chan School of Biomedical Sciences Faculty of MedicineThe Chinese University of Hong KongHong Kong SARChina Kunming Institute of Zoology-The Chinese University of Hong Kong(KIZ-CUHK)Joint Laboratory of Bioresources and Molecular Research of Common Diseases Hong Kong SARChina Hong Kong Branch of CAS Center for Excellence in Animal Evolution and Genetics School of Biomedical SciencesThe Chinese University of Hong KongHong Kong SARChina Key Laboratory for Regenerative Medicine Ministry of EducationSchool of Biomedical SciencesThe Chinese University of Hong KongHong Kong SARChina

Parkinson’s disease(PD)relates to defective mitochondrial quality control in the dopaminergic motor *** studies have revealed that PINK1 and Parkin mutations are indicative of a heightened propensity to PD onset,pinpointing mitophagy and inflammation as the culprit pathways involved in neuronal loss in the substantia nigra(SNpc).In a reciprocal manner,LRRK2 functions in the regulation of basal flux and inflammatory responses responsible for PINK1/Parkin-dependent mitophagy *** intervention in these diseasemodifying pathways may facilitate the development of novel PD therapeutics,despite the current lack of an established drug evaluation *** such,we reviewed the feasibility of employing the versatile global Pink1knockout(KO)rat model as a self-sufficient,spontaneous PD model for investigating both disease etiology and drug *** rats retain clinical features encompassing basal mitophagic flux changes with PD *** demonstrate the versatility of this PD rat model based on the incorporation of additional experimental insults to recapitulate the proinflammatory responses observed in PD patients.

关键词： Parkinson’s disease Mitophagy Inflammatory response Genetic model Pink1 KO rats

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NOD-like receptors mediated activation of eosinophils interacting with bronchial epithelial cells： a link between innate immunity and allergic asthma

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Cellular & Molecular Immunology 2013年第4期10卷 317-329页

作者： Churl Kwok Wong Shuiqing Hu Karen Ming-Lam Leungt Jie Dong Lan He Yi Jun Chu Ida miu-ting Chu Huai-Na Qiu Kelly Yan-Ping Liu ChristopherWai-Kei Lam Department of Chemical Pathology The Chinese University of Hong Kong Prince of Wales Hospital Hong Kong China Department of Medicine and Therapeutics The Chinese University of Hong Kong Prince of Wales Hospital Hong Kong China Macao Institute for Applied Research in Medicine and Health Macao University of Science and Technology Taipa Macao China

Key intracytosolic pattern recognition receptors of innate immunity against bacterial infections are nucleotide-binding oligomerization domain （NOD）-Iike receptors （NLRs）. We elucidated the NOD1 and NOD2-mediated activation of human eosinophils, the principal effector cells for allergic inflammation, upon interacting with human bronchial epithelial BEAS-2B cells in allergic asthma. Eosinophils constitutively expressed NOD1,2 but exhibited nonsignificant responses to release chemokines upon the stimulation by NOD1 ligand 7-D-glutamyl-meso-diaminopimelic acid （iE-DAP） and NOD2 ligand muramyl dipeptide （MDP）. However, iE-DAP and MDP could significantly upregulate cell surface expression of CD18 and intercellular adhesion molecule （ICAM）-I on eosinophils and ICAM-1 on BEAS-2B cells, as well as induce chemokines CCL2 and CXCL8 release in the coculture system （all P〈0.05）. Both eosinophils and BEAS-2B cells were the main source for CXCL8 and CCL2 release in the coculture system upon iE-DAP or MDP stimulation. Direct interaction between eosinophils and BEAS-2B cells is responsible for CCL2 release, and soluble mediators are implicated in CXCL8 release. ERK and NF-KB play regulatory roles for the expression of adhesion molecules and chemokines in coculture. Treatment with NOD1,2 ligand could induce the subepithelial fibrosis and significantly enhance the serum concentration of total IgE, chemokine CCL5 for eosinophils and T helper type 2 （Th2） cells and asthma Th2 cytokine IL-13 in bronchoalveolar lavage fluid of ovalbumin-sensitized allergic asthmatic mice （all P〈0.05）. This study provides further evidence of bacterial infection-mediated activation of NOD1,2 in triggering allergic asthma via the activation of eosinophils interacting with bronchial epithelial cells at inflammatory airway.

关键词： allergy bronchial epithelial cells chemokines eosinophils signal transduction

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