Article
Biochemistry & Molecular Biology
Yovana Cabrera, Ganeko Bernardo-Seisdedos, Leire Dublang, David Albesa-Jove, Natalia Orozco, Ana Rosa Viguera, Oscar Millet, Arturo Muga, Fernando Moro
Summary: This study reveals the role of Apg2 in regulating the activity of human Hsc70, and its C-terminal extension acts as a fine-tuner of substrate remodeling ability in the chaperone system.
JOURNAL OF MOLECULAR BIOLOGY
(2022)
Article
Chemistry, Medicinal
Hao Shao, Xiaokai Li, Shigenari Hayashi, Jeanette L. Bertron, Daniel M. C. Schwarz, Benjamin C. Tang, Jason E. Gestwicki
Summary: The research team synthesized 17 derivatives of YM-08, among which compound JG-23 showed 12-fold better metabolic stability and retained the ability to reduce tau levels in cell models. These chemical probes are expected to be useful tools for studying tau homeostasis.
BIOORGANIC & MEDICINAL CHEMISTRY LETTERS
(2021)
Article
Clinical Neurology
Alessandro Castelli, Fabio Placidi, Chiara Giuseppina Bonomi, Francesca Di Giuliano, Alessandro Martorana, Giulia Pizzicannella, Claudio Liguori, Natalia Manfredi, Luisa Mari, Andrea Pagano, Valentina Bramato, Nicola Biagio Mercuri, Francesca Izzi
Summary: This study evaluated the diagnostic and prognostic value of EEG, MRI, and cerebrospinal fluid features in the diagnostic criteria of CJD. The results showed that periodic sharp wave complexes may identify a distinctive phenotype of CJD characterized by a faster disease progression, shorter survival time, and specific MRI and cerebrospinal fluid features.
CLINICAL NEUROPHYSIOLOGY
(2022)
Article
Virology
Ellen Gelpi, Sigrid Klotz, Nuria Vidal-Robau, Gerda Ricken, Gunther Regelsberger, Thomas Strobel, Ognian Kalev, Marlene Leoni, Herbert Budka, Gabor G. Kovacs
Summary: The study found that in sporadic Creutzfeldt-Jakob disease, the MM2C subtype is more likely to accumulate PrPd in white matter oligodendrocytes, and this pathology is more prominent in patients with a longer disease duration.
Article
Biochemistry & Molecular Biology
Morihiko Nakamura, Yuki Fukuma, Kaori Notsu, Megumi Kono
Summary: Quercetin suppresses nitric oxide production in macrophages and regulates cellular signaling by influencing the action of MNSF beta and HSC70. MNSF beta is involved in TLR4/MyD88 signaling, which is regulated by HSC70 and negatively controlled by quercetin.
MOLECULAR BIOLOGY REPORTS
(2022)
Article
Clinical Neurology
Masahiro Shijo, Motoi Yoshimura, Tsuyoshi Omae, Go Hashimoto, Tadataka Mizoguchi, Takahiro Kuwashiro, Takashi Komori, Yoshio Tsuboi, Tomoko Saito, Masanori Nakagawa, Kyoko Itoh, Hiroyuki Honda
Summary: A study found that patients with V180I gCJD who received PPS treatment exhibited increased PrP deposition and prolonged survival time. This may be attributed to the positive effects of PPS on the aggregation and detoxication of PrP-oligomer.
Article
Cell Biology
Xiaoyan Xu, Yaqin Sun, Xufeng Cen, Bing Shan, Qingwei Zhao, Tingxue Xie, Zhe Wang, Tingjun Hou, Yu Xue, Mengmeng Zhang, Di Peng, Qiming Sun, Cong Yi, Ayaz Najafov, Hongguang Xia
Summary: The study reveals that Metformin can induce CMA through activating the TAK1-IKKα/β signaling pathway to improve the pathogenesis of neurodegenerative diseases. Additionally, Metformin can reduce amyloid-beta plaques in the brain and reverse the molecular and behavioral phenotypes of Alzheimer's disease.
Article
Multidisciplinary Sciences
Francois Kroll, Athanasios Dimitriadis, Tracy Campbell, Lee Darwent, John Collinge, Simon Mead, Emmanuelle Vire
Summary: This study sequenced the full length of PRNP, including its regulatory region, using nanopore sequencing. The accuracy of nanopore sequencing was demonstrated by detecting the same variants as identified by Sanger sequencing, including repeat expansions/deletions. Additional single-nucleotide variants in the non-coding regions of PRNP were identified by nanopore sequencing, but no novel structural variants were discovered. The study also explored somatic mosaicism of PRNP, suggesting that the observed changes may have been generated by the PCR method.
SCIENTIFIC REPORTS
(2022)
Article
Microbiology
Alicia Otero, Tomas Barrio, Hasier Erana, Jorge M. Charco, Marina Betancor, Carlos M. Diaz-Dominguez, Belen Marin, Olivier Andreoletti, Juan M. Torres, Qingzhong Kong, Juan J. Badiola, Rosa Bolea, Joaquin Castilla
Summary: In this study, the effect of glycosylation on the characteristics of BSE isolates was assessed using a non-glycosylated human PrPC-expressing mouse model. The findings showed that BSE prions could be propagated in a non-glycosylated human PrPC environment without losing their strain properties. BSE prions transmitted to the transgenic mouse model exhibited neuropathological and biochemical signs compatible with BSE, confirming that the glycosylation of human PrPC is not essential for the propagation of this particular prion strain.
Article
Multidisciplinary Sciences
Maxime Belondrade, Simon Nicot, Charly Mayran, Lilian Bruyere-Ostells, Florian Almela, Michele A. Di Bari, Etienne Levavasseur, Joel C. Watts, Chantal Fournier-Wirth, Sylvain Lehmann, Stephane Haik, Romolo Nonno, Daisy Bougard
Summary: This study evaluated the amplification of PrPTSE from human sCJD brain samples in different substrates using PMCA, revealing the potential of bank vole PrP substrate in amplifying all sCJD subtypes. In contrast, PMCA in human PrP substrates led to molecular shifts in PrPTSE, with increased permissiveness of V129 PrP substrate to sCJD prion amplification. The combination of PMCA sensitivities and PrPTSE electrophoretic profiles confirmed the classification of 4 distinct major sCJD prion strains. Additionally, the sensitivity required to detect VV2 sCJD prions in cerebrospinal fluid was achieved.
SCIENTIFIC REPORTS
(2021)
Review
Biochemistry & Molecular Biology
Nikol Jankovska, Tomas Olejar, Radoslav Matej
Summary: Neurodegenerative diseases are characterized by the deposition of specific protein aggregates, which can be intracellular or extracellular, depending on the type of disease. Alzheimer's disease is defined by the extracellular plaques composed of amyloid beta-protein, while prionoses refer to plaques formed by amyloid prion protein. Both diseases involve extracellular deposits with signs of neuritic degeneration, indicating a potential role for amyloid protein toxicity.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Biochemistry & Molecular Biology
Igor Tokarchuk, Felice A. Janser, Anna M. Schlafli, Marta Teixeira Pinto, Magali Humbert, Nicolas J. Niklaus, Sabina Berezowska, Rupert Langer, Mario P. Tschan
Summary: High expression of LAMP2A is associated with worse disease-free survival in breast cancer patients with HER2 negative tumors and serves as an independent prognostic parameter. Low levels of LAMP2A can reduce proliferation of breast cancer cell lines.
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS
(2021)
Review
Cell Biology
Yi-Ting Wang, Jia-Hong Lu
Summary: Chaperone-mediated autophagy (CMA) is a protein degradation mechanism associated with neurodegenerative diseases (NDs). Modulating CMA has been shown to promote the degradation of ND-associated proteins and alleviate ND phenotypes, making it a potential therapeutic strategy for ND.
Article
Biochemistry & Molecular Biology
Noeli Soares Melo Silva, Luiz Fernando de Camargo Rodrigues, Paulo Roberto Dores-Silva, Carlos Alberto Montanari, Carlos Henrique Inacio Ramos, Leandro Ramos Souza Barbosa, Julio Cesar Borges
Summary: The study reveals that rHSPA8 shares structural and functional similarities with other human Hsp70s, interacts with adenosine nucleotides, has lower ATPase activity compared to HSPA5/hGrp78/hBiP but higher than recombinant HSPA1A/hHsp70-1A, and shows capacity in preventing the aggregation of client proteins.
BIOCHIMICA ET BIOPHYSICA ACTA-PROTEINS AND PROTEOMICS
(2021)
Article
Microbiology
Jifeng Bian, Sehun Kim, Sarah J. Kane, Jenna Crowell, Julianna L. Sun, Jeffrey Christiansen, Eri Saijo, Julie A. Moreno, James DiLisio, Emily Burnett, Sandra Pritzkow, Damian Gorski, Claudio Soto, Terry J. Kreeger, Aru Balachandran, Gordon Mitchell, Michael W. Miller, Romolo Nonno, Turid Vikoren, Jorn Vage, Knut Madslien, Linh Tran, Tram Thu Vuong, Sylvie L. Benestad, Glenn C. Telling
Summary: The study shows that CWD in Norwegian reindeer and moose is caused by novel prion strains with distinct properties from North American CWD. These Norwegian prions have the potential to produce adapted strains with improved fitness for contagious transmission. This finding has implications for risk analyses and management of emergent European CWD.
Article
Medicine, Research & Experimental
Paul E. Sladen, Pedro R. L. Perdigao, Grace Salsbury, Tatiana Novoselova, Jacqueline van der Spuy, J. Paul Chapple, Patrick Yu-Wai-Man, Michael E. Cheetham
Summary: Autosomal dominant optic atrophy (DOA), the most common inherited optic neuropathy in the United Kingdom, is often caused by variants in the OPA1 gene. Using CRISPR-Cas9 gene editing, it is possible to efficiently correct OPA1 mutations, restoring mitochondrial homeostasis and improving mtDNA integrity in patient-derived cells.
MOLECULAR THERAPY-NUCLEIC ACIDS
(2021)
Correction
Neurosciences
David I. Finkelstein, Jessica L. Billings, Paul A. Adlard, Scott Ayton, Amelia Sedjahtera, Colin L. Masters, Simon Wilkins, David M. Shackleford, Susan A. Charman, Wojciech Bal, Izabela A. Zawisza, Ewa Kurowska, Andrew L. Gundlach, Sheri Ma, Ashley I. Bush, Dominic J. Hare, Philip A. Doble, Simon Crawford, Elisabeth C. L. Gautier, Jack Parsons, Penny Huggins, Kevin J. Barnham, Robert A. Cherny
ACTA NEUROPATHOLOGICA COMMUNICATIONS
(2021)
Article
Surgery
Ali Riaz Baqar, Simon Wilkins, Wei Chun Wang, Karen Oliva, Suellyn Centauri, Raymond Yap, Paul McMurrick
Summary: This study aimed to compare the impact of extracorporeal side-to-side and end-to-side stapled anastomosis on outcomes after an oncological laparoscopic right hemicolectomy. The results showed that the type of stapling technique affected operative duration and postoperative ileus rates, but had no impact on lymph node yield or anastomotic leaks.
ANZ JOURNAL OF SURGERY
(2022)
Article
Biochemistry & Molecular Biology
Paul E. Sladen, Katarina Jovanovic, Rosellina Guarascio, Daniele Ottaviani, Grace Salsbury, Tatiana Novoselova, J. Paul Chapple, Patrick Yu-Wai-Man, Michael E. Cheetham
Summary: Autosomal dominant optic atrophy (DOA) is the most common inherited optic neuropathy, characterized by the preferential loss of retinal ganglion cells (RGCs), resulting in optic nerve degeneration and progressive bilateral central vision loss. There are currently no licensed disease-modifying therapies for DOA and the disease mechanisms driving RGC degeneration are poorly understood.
HUMAN MOLECULAR GENETICS
(2022)
Article
Gastroenterology & Hepatology
Rebekah M. Engel, Thierry Jarde, Karen Oliva, Genevieve Kerr, Wing Hei Chan, Sara Hlavca, David Nickless, Stuart K. Archer, Raymond Yap, Pravin Ranchod, Stephen Bell, Ann Niap, Christine Koulis, Ashley Chong, Simon Wilkins, Trevor C. Dale, Andrew J. Hollins, Paul J. McMurrick, Helen E. Abud
Summary: Patient-derived colorectal cancer organoids (PDCOs) have the potential utility in personalized cancer medicine, as they recapitulate key characteristics of the primary tumor and can be used as a powerful tool for cancer research.
JOURNAL OF GASTROENTEROLOGY AND HEPATOLOGY
(2022)
Article
Surgery
Simon Wilkins, Raymond Yap, Shehara Mendis, Peter Carne, Paul J. McMurrick
Summary: Abdominoperineal resection (APR) of rectal cancer is associated with poorer outcomes than anterior resection. Extra-levator abdominoperineal excision (ELAPE) technique reduced intra-operative perforation (IOP) rates and circumferential resection margin (CRM) involvement, but increased wound complications. Studies suggest that sub-optimal surgical technique may be responsible for high CRM and IOP rates rather than the technique itself. ELAPE does not always have lower CRM involvement rates than APR and has higher morbidity. The impact of ELAPE on oncological outcomes is still uncertain.
FRONTIERS IN SURGERY
(2022)
Article
Oncology
Hengkang Yan, Mary E. Vail, Linda Hii, Nancy Guo, Paul J. McMurrick, Karen Oliva, Simon Wilkins, Nayanendu Saha, Dimitar B. Nikolov, Fook-Thean Lee, Andrew M. Scott, Peter W. Janes
Summary: ADAM10 is a cell surface protein that can control functions during normal development and become deregulated in cancer cells. Researchers developed an antibody that can detect the active form of ADAM10 and successfully used it to kill tumor cells.
Review
Biochemistry & Molecular Biology
Kalliopi Ziaka, Jacqueline van der Spuy
Summary: This review explores the role of Hsp90 in the proteostatic mechanisms of photoreceptors and elaborates on its function when retinal homeostasis is disturbed. It also discusses the impact of Hsp90 on retinal diseases through its interaction with specific client proteins.
Article
Cell & Tissue Engineering
Amy Leung, Almudena Sacristan-Reviriego, Pedro R. L. Perdigao, Hali Sai, Michalis Georgiou, Angelos Kalitzeos, Amanda -Jayne F. Carr, Peter J. Coffey, Michel Michaelides, James Bainbridge, Michael E. Cheetham, Jacqueline van der Spuy
Summary: Leber congenital amaurosis type 4 (LCA4) is a severe sight impairment disease caused by AIPL1 mutations. Researchers used induced pluripotent stem cells (iPSCs) derived from renal epithelial cells to generate retinal organoids and found that the photoreceptor cells in these organoids exhibited the characteristics of LCA4. They also discovered a potential therapeutic drug, but its ability to restore the photoreceptor cells was limited.
Article
Surgery
Anna Taylor, Simon Wilkins, Nicholas Gelber, Wei Chun Wang, Karen Oliva, Raymond Yap, Paul McMurrick, Pravin Ranchod
Summary: The aim of this study was to assess the efficacy of intravenous hyoscine butylbromide on the accuracy of MRI rectal cancer staging. The results showed that while IV hyoscine butylbromide improved the accuracy of T2 and T3 staged rectal cancer, it had no effect on T1 staged cancer or N-staging. Therefore, department protocols may need to be re-assessed regarding the prescription of these medications for MRI rectal cancer staging.
ANZ JOURNAL OF SURGERY
(2023)
Review
Biochemistry & Molecular Biology
Daniela Benati, Amy Leung, Pedro Perdigao, Vasileios Toulis, Jacqueline van der Spuy, Alessandra Recchia
Summary: Inherited retinal disorders (IRDs) impact a large number of individuals worldwide and are a significant cause of irreversible blindness. CRISPR/Cas technology, a fast-evolving gene-editing tool, has emerged as a potential treatment for retinal degenerative diseases. It has been extensively used in ophthalmic studies, both for in vivo proof of gene therapies and for disease modeling in basic research. The technology has been applied to genetically modify human induced pluripotent stem cells (iPSCs) to accurately recapitulate IRD mechanisms for the development of treatments and to provide a cell source for autologous transplantation.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Surgery
Christine Georges, Raymond Yap, Stephen Bell, Keith Chip Farmer, Lauren C. L. Cohen, Simon Wilkins, Suellyn Centauri, Rebekah Engel, Karen Oliva, Paul J. McMurrick
Summary: This retrospective study evaluated the impact of four colorectal surgical procedures on patient-reported outcome measures. The study found that patients who underwent a right hemicolectomy had the best post-operative function and reduced symptoms, while patients who underwent an ultra-low anterior resection or an abdominoperineal resection had the worst outcomes.
ANZ JOURNAL OF SURGERY
(2023)
Review
Oncology
Rezav Amiri Khosroshahi, Mohammad Heidari Seyedmahalle, Sheida Zeraattalab-Motlagh, Laleh Fakhr, Simon Wilkins, Hamed Mohammadi
Summary: This study aimed to investigate the dose-dependent effects of omega-3 fatty acids supplementation on inflammatory factors in cancer patients. The results showed that daily intake of 1 g of omega-3 fatty acids (oral/enteral) significantly reduced IL-6 and TNF-alpha levels. Intake of 0.5 g/kg/day of omega-3 fatty acids (parenteral) also had a positive impact on TNF-alpha, but with low certainty of evidence.
NUTRITION AND CANCER-AN INTERNATIONAL JOURNAL
(2023)
Article
Nutrition & Dietetics
Reza Amiri Khosroshahi, Hamed Mohammadi, Maryam Barkhordar, Sheida Zeraattalab-Motlagh, Hossein Imani, Amirabbas Rashidi, Erfan Sadeghi, Simon Wilkins, Seyed Asadollah Mousavi
Summary: This study compared nutritional screening tools for patients with leukemia before allo-HSCT and found that there was good agreement between GLIM and NRS-2002, fair agreement between GLIM and ESPEN, and fair agreement between NRS-2002 and ESPEN. There was moderate agreement among all the tools. NRS-2002 is an accepted tool for screening malnutrition and showed good agreement with GLIM in this study. Further research is needed to introduce a valid tool in the HSCT setting.
FRONTIERS IN NUTRITION
(2023)
Article
Gastroenterology & Hepatology
Raymond Yap, Simon Wilkins, Mohammad Asghari-Jafarabadi, Karen Oliva, Wei Chun Wang, Suellyn Centauri, Paul J. McMurrick
Summary: This study aims to investigate the postoperative outcomes of colorectal cancer surgery in patients aged 80 and over. The results demonstrate that surgical treatment of older patients is safe with acceptable survival rates. However, efforts are needed to reduce complications in older patients.
INTERNATIONAL JOURNAL OF COLORECTAL DISEASE
(2023)
Article
Biochemistry & Molecular Biology
Kun He, Mengyi Xie, Weifeng Hong, Yonghe Li, Yaolin Yin, Xiaojin Gao, Yi He, Yu Chen, Chuan You, Jingdong Li
Summary: Centromere protein L (CENPL) is overexpressed in hepatocellular carcinoma (HCC) and is associated with poor patient prognosis. Upregulation of CENPL promotes tumor proliferation and glycolysis in HCC cells by activating the MEK1/2-ERK1/2 signaling pathway.
INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY
(2024)
Article
Biochemistry & Molecular Biology
Yingzi Wang, Haozhong Huang, Huimin Weng, Chunsen Jia, Bin Liao, Yang Long, Fengxu Yu, Yongmei Nie
Summary: Talin protein plays a crucial role in transmitting mechanical forces by connecting the extracellular matrix to the cytoskeleton. It converts mechanical signals into biochemical signals and serves as diagnostic, therapeutic, and prognostic indicators in diseases.
INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY
(2024)
Review
Biochemistry & Molecular Biology
Hema Saranya Ilamathi, Marc Germain
Summary: Mitochondria are the central metabolic hubs in cells, relying on proteins encoded by nuclear DNA and mitochondrial DNA (mtDNA) for their function. The maintenance and distribution of mtDNA are crucial for proper mitochondrial function and are associated with mitochondrial diseases. mtDNA is organized into nucleoids that dynamically move and interact with each other. The replication and distribution of mtDNA nucleoids are regulated by the complex interplay of mitochondrial dynamics, ER-mitochondria contact sites, and cytoskeletal networks.
INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY
(2024)
Article
Biochemistry & Molecular Biology
K. K. Sruthi, Sirisha Natani, Ramesh Ummanni
Summary: The overexpression of TPD52 is associated with the emergence of neuroendocrine prostate cancer (NEPC). TPD52 activates the NF-kappa B - STAT3 axis to induce neuroendocrine differentiation (NED) of prostate cancer cells. Therapeutic targeting of TPD52 is important for the treatment of prostate cancer.
INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY
(2024)
Article
Biochemistry & Molecular Biology
Cui Li, Feifan Xiang, Yuqi Gong, Yi Fu, Ge Chen, Zhi Wang, Zhong Li, Daiqing Wei
Summary: Our study demonstrates the significant role of tumor-derived microparticles (T-MPs) in osteosarcoma metastasis and immune response. T-MPs promote macrophage polarization and stimulate migration and chemoresistance of osteosarcoma cells, thereby affecting the progression of osteosarcoma.
INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY
(2024)