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12. Diagnostic Potential of Alternations of Bile Acid Profiles in the Plasma of Patients with Huntington's Disease.

Author

Chiang, Ping-I, Chang, Kuo-Hsuan, Tang, Hsiang-Yu, Wu, Yih-Ru, Cheng, Mei-Ling, and Chen, Chiung-Mei

Subjects
HUNTINGTON disease, BILE acids, CHOLESTEROL metabolism, URSODEOXYCHOLIC acid, METABOLIC disorders
Abstract

Huntington's disease (HD) is characterized by progressive involuntary chorea movements and cognitive decline. Recent research indicates that metabolic disturbance may play a role in its pathogenesis. Bile acids, produced during cholesterol metabolism in the liver, have been linked to neurodegenerative conditions. This study investigated variations in plasma bile acid profiles among individuals with HD. Plasma levels of 16 primary and secondary bile acids and their conjugates were analyzed in 20 healthy controls and 33 HD patients, including 24 with symptoms (symHD) and 9 carriers in the presymptomatic stage (preHD). HD patients exhibited significantly higher levels of glycochenodeoxycholic acid (GCDCA) and glycoursodeoxycholic acid (GUDCA) compared to healthy controls. Conversely, isolithocholic acid levels were notably lower in the HD group. Neurotoxic bile acids (glycocholic acid (GCA) + glycodeoxycholic acid (GDCA) + GCDCA) were elevated in symHD patients, while levels of neuroprotective bile acids (ursodeoxycholic acid (UDCA) + GUDCA + tauroursodeoxycholic acid (TUDCA)) were higher in preHD carriers, indicating a compensatory response to early neuronal damage. These results underscore the importance of changes in plasma bile acid profiles in HD and their potential involvement in disease mechanisms. The identified bile acids (GCDCA, GUDCA, and isolithocholic acid) could potentially serve as markers to distinguish between HD stages and healthy individuals. Nonetheless, further research is warranted to fully understand the clinical implications of these findings and their potential as diagnostic or therapeutic tools for HD. [ABSTRACT FROM AUTHOR]

Published
2024
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15. Identification of Blood Metabolic biomarkers associated with diabetic distal symmetric sensorimotor polyneuropathy in patients with Type 2 Diabetes Mellitus

Author

Chang, Kuo‐Hsuan, primary, Chen, Chiung‐Mei, additional, Lin, Chia‐Ni, additional, Tsai, Sung‐Sheng, additional, Lyu, Rong‐Kuo, additional, Chu, Chun‐Che, additional, Ro, Long‐Sun, additional, Liao, Ming‐Feng, additional, Chang, Hong‐Shiu, additional, Weng, Yi‐Ching, additional, Hwang, Jawl‐Shan, additional, and Kuo, Hung‐Chou, additional

Published
2023
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21. Coumarin-chalcone hybrid LM-021 and indole derivative NC009-1 targeting inflammation and oxidative stress to protect BE(2)-M17 cells against α-synuclein toxicity

Author

Yang, Pei-Ning, primary, Chen, Wan-Ling, additional, Lee, Jun-Wei, additional, Lin, Chih-Hsin, additional, Chen, Yi-Ru, additional, Lin, Chung-Yin, additional, Lin, Wenwei, additional, Yao, Ching-Fa, additional, Wu, Yih-Ru, additional, Chang, Kuo-Hsuan, additional, Chen, Chiung-Mei, additional, and Lee-Chen, Guey-Jen, additional

Published
2023
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27. Investigating the functional consequences of expanded triplet repeat sequence in a mouse model of Huntington's Disease (HD)

Author

Chen, Chiung-Mei

Subjects
616.851027, QH301 Biology, QH426 Genetics
Abstract

A PCR strategy showed that a number of total mtDNA molecules was significantly decreased (~30%) in the striatum (no reduction in the cortex and cerebellum) of 24-month old HD mice, but not a 15 months of age, when compared to wild-type mice, suggesting mtDNA depletion is a progressive rather than a developmental phenomenon. In light of the ~30% reduction of total mtDNA in the striatum, expression levels of the mitochondrial DNA-encoded respiratory complex enzymes, cytochrome b(Cytb), cytochrome c oxidase I (COI) and cytochrome c oxidase II (COII) were investigated in different brain regions of HD mice. At ~25 months of age, there were no significant differences in mRNA levels of CoII and Cytb in any brain region (striatum, cortex and cerebellum) studied when compared to normal littermates. However, HD mice showed significantly decreased CO-I protein levels and marginally decreased CoI mRNA levels in the striatum. Reduced levels of mtDNA may be caused by decreased replication of mtDNA or increased oxidative damage of mtDNA. Increased levels of 8-OHdG, a marker of increased oxidative stress, were detected in the dorsomedial, dorsolateral and ventromedial striatum, but not in the cortex of 24-month old HD mice providing direct evidence that increased oxidative stress specifically occurs in the striatum of HD mice. As no alterations in the mitochondrial transcription factor (mtTFA) in the striatum of HD mice could be detected, it is likely that mtDNA depletion in the HD mice is caused by increased levels of oxidative stress rather than decreased replication. The results provide a basis for further studies investigating how mutant huntingtin causes increased levels of oxidative stress and for identifying novel therapeutic targets.

Published
2002

28. Diagnostic and Therapeutic Challenges

Author

Huang, Chu-Yen, Kang, Eugene Yu-Chuan, Kuo, Hung-Chou, Chen, Chiung-Mei, Lo, Fu-Sung, Yeh, Lung-Kun, Chen, Kuan-Jen, Wang, Nan-Kai, Takahashi, Vitor K.L., Xu, Christine L., and Tsang, Stephen H.

Published
2020
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30. Investigating Therapeutic Effects of Indole Derivatives Targeting Inflammation and Oxidative Stress in Neurotoxin-Induced Cell and Mouse Models of Parkinson’s Disease

Author

Chiu, Ya-Jen, primary, Lin, Chih-Hsin, additional, Lin, Chung-Yin, additional, Yang, Pei-Ning, additional, Lo, Yen-Shi, additional, Chen, Yu-Chieh, additional, Chen, Chiung-Mei, additional, Wu, Yih-Ru, additional, Yao, Ching-Fa, additional, Chang, Kuo-Hsuan, additional, and Lee-Chen, Guey-Jen, additional

Published
2023
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41. Increased 18 F-FDG uptake in denervated muscles in a case of Parsonage-Turner syndrome.

Author

Chiang, Ping-I and Chen, Chiung-Mei

Subjects
*BRACHIAL plexus neuropathies, *POSITRON emission tomography computed tomography, *MAGNETIC resonance neurography, *PERIPHERAL nerve injuries, *NERVE conduction studies, *RADICULOPATHY
Abstract

Background: Parsonage-Turner Syndrome (PTS) is a rare brachial plexopathy characterized by the sudden onset of pain in the shoulder girdle followed by upper limb weakness. PTS is frequently under-recognized or misdiagnosed as other more common neurological disorders presenting in a similar fashion, such as cervical radiculopathy which may require surgical intervention. Accurate diagnosis and prompt management implicate a good prognosis. Although electrophysiological studies are considered the most important for evaluating peripheral nerve injuries, it usually takes time, up to 3 weeks after the initial insult of the nerve for electromyogram (EMG) and nerve conduction studies (NCS) to display abnormalities. In the cases of PTS, especially when initial EMG/NCS and magnetic resonance neurography (MRN) results are inconclusive, 18 F-FDG positron emission tomography and computed tomography (18 F-FDG PET-CT) may be useful in helping the early detection of muscle denervation. Case presentation: A 60-year-old right-handed Taiwanese woman presented with sudden onset of intense and sharp left shoulder girdle pain without radiating to the arm, followed by muscle weakness of her left arm in abduction and elevation 3 days after the onset of pain. A detailed neurological examination and EMG and NCS suggested the clinical diagnosis of left brachial plexopathy. MRN imaging revealed no significant abnormality. 18 F-FDG PET-CT showed increased uptake in denervated muscles (supraspinatus, deltoid, and biceps muscles). Treatment with oral prednisolone and physiotherapy significantly improved pain and muscle weakness. Conclusions: We present increased 18 F-FDG uptake in denervated muscles detected by 18 F-FDG PET-CT. 18 F-FDG PET-CT may serve as an adjunct examination to evaluate PTS, which has been suggested previously but rarely reported. [ABSTRACT FROM AUTHOR]

Published
2023
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47. Medicinal herbs Oerianthe javanica (Blume) DC., Casuarina equisetifolia L. and Sorghum bicolor (L.) Moench protect human cells from [MPP.sup.+] damage via inducing FBX07 expression

Author

Chen, Chiung-Mei, Chen, I-Cheng, Chen, Ying-Lin, Lin, Te-Hsien, Chen, Wan-Ling, Chao, Chih-Ying, Wu, Yih-Ru, Lu, Yeah-Ting, Lee, Cheng-Yu, Chien, Hong-Chi, Chen, Ting-Shou, Lee-Chen, Guey-Jen, and Lee, Chi-Mei

Subjects
Gene expression -- Health aspects, Nervous system diseases -- Care and treatment, Materia medica, Vegetable -- Usage -- Health aspects, Dopaminergic mechanisms -- Genetic aspects -- Health aspects, Medicinal plants -- Usage -- Health aspects, Plant extracts -- Usage -- Health aspects, Biological sciences, Health, Science and technology
Abstract

ABSTRACT Background: The F-box protein 7 (FBX07) mutations have been identified in families with early-onset parkinsonism and pyramidal tract signs, and designated as PARK15. In addition, FBX07 mutations were found [...]

Published
2016
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50. Establishment of a new classification system for chronic inflammatory demyelinating polyneuropathy based on unsupervised machine learning

Author

Chang, Chun‐Wei, primary, Ro, Long‐Sun, additional, Lyu, Rong‐Kuo, additional, Kuo, Hung‐Chou, additional, Liao, Ming‐Feng, additional, Wu, Yih‐Ru, additional, Chen, Chiung‐Mei, additional, Chang, Hong‐Shiu, additional, Weng, Yi‐Ching, additional, Huang, Chin‐Chang, additional, and Chang, Kuo‐Hsuan, additional

Published
2022
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