Article
Neurosciences
Marina Betancor, Laura Moreno-Martinez, Oscar Lopez-Perez, Alicia Otero, Adelaida Hernaiz, Tomas Barrio, Juan Jose Badiola, Rosario Osta, Rosa Bolea, Inmaculada Martin-Burriel
Summary: TTC, as a neuroprotective molecule, shows significant neuroprotective effects in murine models of prion diseases, reducing apoptosis, regulating autophagy, and increasing neuronal survival, although it does not extend survival time in these models.
MOLECULAR NEUROBIOLOGY
(2021)
Review
Clinical Neurology
Lindsey B. De Lott, Jeffrey L. Bennett, Fiona Costello
Summary: Optic neuritis is an inflammatory optic neuropathy that is often associated with demyelinating disorders of the central nervous system. However, it is frequently misdiagnosed in clinical practice, leading to inappropriate treatment or delays in diagnosis. With an increased understanding of immunopathology, novel disease management strategies are emerging to minimize vision loss and improve long-term surveillance.
JOURNAL OF NEUROLOGY
(2022)
Review
Biochemistry & Molecular Biology
Yasushi Kitaoka, Kana Sase
Summary: The optic nerve consists of various components including glia, vessels, and axons. Understanding the molecular mechanisms of optic nerve degeneration can be aided by studying axonal degeneration models. Autophagy plays a crucial role in this process, and its impairment may lead to axonal degeneration. Enhancing autophagy can potentially protect the axons and prevent further cell death.
MOLECULAR ASPECTS OF MEDICINE
(2023)
Review
Pharmacology & Pharmacy
Rosa M. Coco-Martin, Salvador Pastor-Idoate, Jose Carlos Pastor
Summary: Cell therapy holds great promise for treating retinal and optic nerve diseases, but there are challenges to overcome such as inhibition of cell proliferation and differentiation in vitro and limited long-term survival and functioning of transplanted cells. Clinical translation of cell-based therapy also faces issues like enrichment for specific retinal subtypes, cell survival, cell delivery methods, and the risk of tumor formation from undifferentiated stem cells. Despite these challenges, stem/progenitor cells remain the most promising strategy for future treatment of retinal and optic nerve diseases.
Article
Neurosciences
Natallia Makarava, Olga Mychko, Jennifer Chen-Yu Chang, Kara Molesworth, Ilia Baskakov
Summary: The study found that in neurodegenerative diseases, the degree of astrocyte activation and disturbance in physiological pathways may have a significant impact on the rapid progression of the disease.
ACTA NEUROPATHOLOGICA COMMUNICATIONS
(2021)
Article
Biochemistry & Molecular Biology
Alberto Rabano, Carmen Guerrero Marquez, Ramon A. Juste, Maria V. Geijo, Miguel Calero
Summary: The study on the lesion profile of medial temporal lobe in human prion disease patients revealed diverse profiles for different types and subtypes of HPD, with mixed tissue types showing a higher impact on MTL lesion profile. These results may contribute to the analysis of possible strain effects in focal non-prion neurodegenerative conditions limited to the MTL.
Article
Biochemistry & Molecular Biology
Rosalia Bruno, Geraldina Riccardi, Floriana Iacobone, Flavia Chiarotti, Laura Pirisinu, Ilaria Vanni, Stefano Marcon, Claudia D'Agostino, Matteo Giovannelli, Piero Parchi, Umberto Agrimi, Romolo Nonno, Michele Angelo Di Bari
Summary: This study investigated the relationship between prion strains and astrocyte phenotype in six human- and animal-vole-adapted strains. The results showed that astrocyte morphology and astrocyte-associated PrPSc deposition varied depending on the strain, indicating strain-specific phenotypes of reactive astrocytes. Additionally, astrocyte-associated PrPSc deposition was observed in four out of six strains and correlated with astrocyte size.
Article
Cell Biology
Meysam Yazdankhah, Sayan Ghosh, Haitao Liu, Stacey Hose, J. Samuel Zigler, Debasish Sinha
Summary: Mitochondrial dysfunction in astrocytes is linked to the development of neurological disorders, and efficient mitophagy in these cells is crucial for the health and functional integrity of the optic nerve.
Article
Clinical Neurology
Zhongyun Chen, Yu Kong, Jing Zhang, Min Chu, Li Liu, Kexin Xie, Yue Cui, Hong Ye, Junjie Li, Lin Wang, Liyong Wu
Summary: The study aimed to assess the proportion of MM2-type sCJD and describe the clinical features of MM2C and MM2T-type sCJD in a Chinese cohort. Among the 209 patients with sCJD, 24.4% were diagnosed with MM2-type sCJD, with 44 cases of MM2C-type and 7 cases of MM2T-type. The absence of typical sCJD symptoms within 6 months was found in 61.3% of MM2C-type sCJD patients on admission, but all had cortical hyperintensity on DWI. MM2C-type sCJD was associated with slower disease progression and the absence of typical clinical features, while MM2T-type had distinct characteristics such as higher male proportion, earlier age of onset, longer disease duration, and higher incidence of bilateral thalamic hypometabolism/hypoperfusion.
ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY
(2023)
Review
Biochemistry & Molecular Biology
Adelaida Hernaiz, Janne Markus Toivonen, Rosa Bolea, Inmaculada Martin-Burriel
Summary: Prion diseases and prion-like diseases involve protein misfolding and aggregation, with the potential involvement of epigenetic mechanisms in the pathology of these diseases. These findings could be important clues for disease pathology research and biomarker development.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Cell Biology
Meysam Yazdankhah, Sayan Ghosh, Peng Shang, Nadezda Stepicheva, Stacey Hose, Haitao Liu, Xitiz Chamling, Shenghe Tian, Mara L. G. Sullivan, Michael Joseph Calderon, Christopher S. Fitting, Joseph Weiss, Ashwath Jayagopal, James T. Handa, Jose-Alain Sahel, J. Samuel Zigler, Paul R. Kinchington, Donald J. Zack, Debasish Sinha
Summary: The differentiation of oligodendrocyte lineage cells involves autophagy and mitochondrial dynamics, with BNIP3L playing a crucial role in the regulation of mitochondrial function and cell viability. Targeting BNIP3L-mediated mitophagy could be a novel therapeutic approach for promoting myelin repair in optic nerve demyelinating diseases.
Article
Biochemistry & Molecular Biology
Casey J. Keuthan, Julie A. Schaub, Meihan Wei, Weixiang Fang, Sarah Quillen, Elizabeth Kimball, Thomas V. Johnson, Hongkai Ji, Donald J. Zack, Harry A. Quigley
Summary: The level of intraocular pressure (IOP) is a major risk factor for glaucomatous optic neuropathy. This study found that the unmyelinated optic nerve region is differentially susceptible to IOP-induced damage compared to the myelinated region. Gene expression patterns in the unmyelinated optic nerve showed significant differences compared to the myelinated optic nerve and retina. Changes in gene expression induced by optic nerve injury were more extensive in the myelinated region than the unmyelinated region.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Neurosciences
An Beckers, Sophie Vanhunsel, Annelies Van Dyck, Steven Bergmans, Luca Masin, Lieve Moons
Summary: Optic neuropathies are disorders that damage the axons of retinal ganglion cells, leading to visual impairment or blindness with no current efficient treatment. Studying optic nerve damage in adult zebrafish revealed enhanced autophagy, suggesting modulation of autophagy could be a therapeutic method to induce central nervous system regeneration.
Article
Veterinary Sciences
Woo-Sung Jo, Yong-Chan Kim, Jae-Ku Oem, Byung-Hoon Jeong
Summary: This study reports novel single nucleotide polymorphisms in the PRNP gene of raccoon dogs and identifies high homology between the PrP of raccoon dogs and dogs, which are prion-resistant animals. Raccoon dog PrP-specific amino acids are related to low amyloid propensity and inherent characteristics of raccoon dog PrP's 3D structure compared to prion-susceptible species.
FRONTIERS IN VETERINARY SCIENCE
(2022)
Article
Biochemistry & Molecular Biology
Rocio Benitez-Fernandez, Carolina Melero-Jerez, Carmen Gil, Enrique J. de la Rosa, Ana Martinez, Fernando de Castro
Summary: This study validates the use of optical coherence tomography (OCT) to assess remyelination in multiple sclerosis (MS) patients and confirms the therapeutic potential of the small molecule VP3.15 as a neuroprotective, anti-inflammatory, and potentially remyelinating drug for MS therapy. The results emphasize the relevance of OCT for monitoring the efficacy of remyelinating therapies in vivo and highlight VP3.15 as a potential disease modifying drug for MS therapy.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Clinical Neurology
Agata Gajos, Janusz Dabrowski, Malgorzata Bienkiewicz, Anna Plachcinska, Jacek Kusmierek, Andrzej Bogucki
NEUROLOGIA I NEUROCHIRURGIA POLSKA
(2019)
Letter
Clinical Neurology
Marija Dulovic-Mahlow, Agata Gajos, Hauke Baumann, Jelena Pozojevic, Frank J. Kaiser, Andrzej Bogucki, Katja Lohmann
PARKINSONISM & RELATED DISORDERS
(2019)
Article
Clinical Neurology
F. Llorens, A. Villar-Pique, M. Schmitz, D. Diaz-Lucena, M. Wohlhage, P. Hermann, S. Goebel, I Schmidt, M. Glatzel, J-J Hauw, B. Sikorska, P. P. Liberski, J. Riggert, I Ferrer, I Zerr
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY
(2020)
Article
Clinical Neurology
Wolfgang H. Jost, Andrzej Friedman, Olaf Michel, Christian Oehlwein, Jaroslaw Slawek, Andrzej Bogucki, Stanislaw Ochudlo, Marta Banach, Fernando Pagan, Birgit Flatau-Baque, Ulrike Dorsch, Janos Csikos, Andrew Blitzer
PARKINSONISM & RELATED DISORDERS
(2020)
Article
Clinical Neurology
Joanna Siuda, Magdalena Boczarska-Jedynak, Slawomir Budrewicz, Jaroslaw Dulski, Monika Figura, Urszula Fiszer, Agata Gajos, Agnieszka Gorzkowska, Ewa Koziorowska-Gawron, Dariusz Koziorowski, Anna Krygowska-Wajs, Monika Rudzinska-Bar, Jaroslaw Slawek, Xuehan Ren, Sheng Luo, Pablo Martinez-Martin, Glenn Stebbins, Christopher G. Goetz, Grzegorz Opala, Andrzej Bogucki, Piotr Janik, Magdalena Koszewicz, Marta Lenska-Mieciek, Malgorzata Michalowska, Marta Piascik-Gromada, Katarzyna Potasz-Kulikowska, Marek Smilowski, Anna Wasielewska, Magdalena Wojcik-Pedziwiatr
NEUROLOGIA I NEUROCHIRURGIA POLSKA
(2020)
Article
Clinical Neurology
Emma Jones, Holger Hummerich, Emmanuelle Vir, James Uphill, Athanasios Dimitriadis, Helen Speedy, Tracy Campbell, Penny Norsworthy, Liam Quinn, Jerome Whitfield, Jacqueline Linehan, Zane Jaunmuktane, Sebastian Brandner, Parmjit Jat, Akin Nihat, Tze How Mok, Parvin Ahmed, Steven Collins, Christiane Stehmann, Shannon Sarros, Gabor G. Kovacs, Michael D. Geschwind, Aili Golubjatnikov, Karl Frontzek, Herbert Budka, Adriano Aguzzi, Hata Karamujic-Comic, Sven J. van der Lee, Carla A. Ibrahim-Verbaas, Cornelia M. van Duijn, Beata Sikorska, Ewa Golanska, Pawel P. Liberski, Miguel Calero, Olga Calero, Pascual Sanchez-Juan, Antonio Salas, Federico Martinon-Torres, Elodie Bouaziz-Amar, Stephane Haik, Jean-Louis Laplanche, Jean-Phillipe Brandel, Phillipe Amouyel, Jean-Charles Lambert, Piero Parchi, Anna Bartoletti-Stella, Sabina Capellari, Anna Poleggi, Anna Ladogana, Maurizio Pocchiari, Serena Aneli, Giuseppe Matullo, Richard Knight, Saima Zafar, Inga Zerr, Stephanie Booth, Michael B. Coulthart, Gerard H. Jansen, Katie Glisic, Janis Blevins, Pierluigi Gambetti, Jiri Safar, Brian Appleby, John Collinge, Simon Mead
Article
Cell Biology
Monika Witusik-Perkowska, Dariusz J. Jaskolski, Pawel P. Liberski, Janusz Szemraj
Summary: This study examines the expression profile of specific lncRNAs involved in the tumor resistance network in glioblastoma-derived models. The results show significant divergence in lncRNA expression between in vivo and in vitro models, and these lncRNAs influence tumor resistance through various processes. Furthermore, differences in the levels of potential lncRNA targets were identified between in vivo and in vitro models, suggesting the importance of considering the microenvironment in drug response testing.
CELLULAR AND MOLECULAR NEUROBIOLOGY
(2022)
Article
Biochemistry & Molecular Biology
Renata Gruszka, Krzysztof Zakrzewski, Pawel Piotr Liberski, Magdalena Zakrzewska
Summary: The study investigated the expression of components of the MYC/E2F/miR-17-92 network in common childhood brain tumors, showing a positive correlation between gene expression and tumor grade and type. Most members of miR-17-92, miR-106a-363, and miR-106b-25 clusters were upregulated, with the highest expression noted for miR-18a and miR-18b. miRNA expression also depended on tumor grade and histology.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Medicine, General & Internal
Jaroslaw Fabis, Marian Danilewicz, Kryspin R. Niedzielski, Michal Waszczykowski, Anna Fabis-Strobin, Andrzej Bogucki
Summary: The study investigated the reversibility of muscle fatty infiltration in a rabbit model of rotator cuff tear, finding that it can be substantially reversed by eccentric training despite subclinical impairment of neuromuscular transmission. Additionally, a 0.63% reversal of MFI was correlated with 1% hypertrophy of type I and II muscle fiber diameter.
ARCHIVES OF MEDICAL SCIENCE
(2021)
Article
Clinical Neurology
Joanna Siuda, Magdalena Boczarska-Jedynak, Slawomir Budrewicz, Jaroslaw Dulski, Monika Figura, Urszula Fiszer, Agata Gajos, Agnieszka Gorzkowska, Ewa Koziorowska-Gawron, Dariusz Koziorowski, Anna Krygowska-Wajs, Monika Rudzinska-Bar, Jaroslaw Slawek, Mateusz Tos, Magdalena Wojcik-Pedziwiatr, Jeffrey Lin, Sheng Luo, Pablo Martinez-Martin, Glenn T. Stebbins, Christopher G. Goetz, Grzegorz Opala
Summary: The study validated the Polish translation of the UDysRS and found that it shared a consistent factor structure with the Reference Standard Spanish version. The Official Polish UDysRS translation is recommended for use in clinical and research settings, providing a common ground for studying disease manifestations and progression across cultures.
NEUROLOGIA I NEUROCHIRURGIA POLSKA
(2021)
Review
Clinical Neurology
Halina Car, Andrzej Bogucki, Marcin Bonikowski, Malgorzata Dec-Cwiek, Artur Druzdz, Dariusz Koziorowski, Monika Rudzinska-Bar, Iwona Sarzynska-Dlugosz, Jaroslaw Slawek
Summary: The three different formulations of Botulinum neurotoxin type-A share a common mechanism of action, but demonstrate significant differences in structure, pharmacological properties, and bioequivalence.
NEUROLOGIA I NEUROCHIRURGIA POLSKA
(2021)
Review
Clinical Neurology
Jaroslaw Slawek, Andrzej Bogucki, Marcin Bonikowski, Halina Car, Malgorzata Dec-Cwiek, Artur Druzdz, Dariusz Koziorowski, Iwona Sarzynska-Dlugosz, Monika Rudzinska
Summary: This paper reviews direct comparisons of the three major BoNT/A preparations on the market, showing how effective doses are adopted in well-established indications, but no universal conversion ratios have been established. All preparations should be treated as distinct medications with respect to their summary of product characteristics when used in everyday practice.
NEUROLOGIA I NEUROCHIRURGIA POLSKA
(2021)
Review
Neurosciences
Renata Gruszka, Krzysztof Zakrzewski, Pawel P. Liberski, Magdalena Zakrzewska
FOLIA NEUROPATHOLOGICA
(2020)