Article
Cell Biology
Michael Kittl, Martina Winklmayr, Julia Preishuber-Pfluegl, Victoria Strobl, Martin Gaisberger, Markus Ritter, Martin Jakab
Summary: Acidification can protect articular chondrocytes from apoptosis, and the inhibition of Cl- currents plays a significant role in this process.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2022)
Article
Biology
Tomohiro Numata, Kaori Sato-Numata, Meredith C. Hermosura, Yasuo Mori, Yasunobu Okada
Summary: Animal cells can regulate their volume after swelling through the regulatory volume decrease (RVD) mechanism, in which TRPM7 plays a crucial role in enhancing VSOR activity and LRRC8A expression. TRPM7 facilitates VSOR activity by increasing the expression of LRRC8A mRNA and stabilizing the plasmalemmal expression of LRRC8A protein.
COMMUNICATIONS BIOLOGY
(2021)
Article
Cell Biology
Eric E. Figueroa, Jerod S. Denton
Summary: The study identified zinc pyrithione (ZPT) as a potent activator of VRAC, shedding light on its mechanism of action. This discovery highlights the utility of high-throughput screening in identifying small-molecule modulators of VRAC and expands the repertoire of pharmacological tool compounds for probing the physiology and regulation of this important channel.
AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY
(2021)
Review
Geriatrics & Gerontology
Zhiqin Deng, Xiaoqiang Chen, Zicong Lin, Murad Alahdal, Daping Wang, Jianquan Liu, Wencui Li
Summary: This review focuses on the important role of volume control-related ion channels in cartilage matrix remodeling and summarizes current views. Additionally, the potential mechanism of the volume-sensitive anion channel LRRC8A in the early occurrence of osteoarthritis is discussed.
Article
Oncology
Xiurou Yang, Chao Li, Xuzhen Liao, Shiqing Liu, Xue Li, Xiuying Hou, Kai Wang, Haifeng Yang, Lvfen Gao, Linyan Zhu
Summary: This study verified for the first time that PTX can induce pyroptosis-like cell death in ovarian cancer cells, and this effect is mediated by the inhibition of chloride channels. It was also found that the combination of PTX and VRACs blockers can enhance the pyroptosis-inducing effect of PTX, providing a potential approach for ovarian cancer chemotherapy.
Article
Biophysics
Benjamin Koenig, Tobias Stauber
BIOPHYSICAL JOURNAL
(2019)
Article
Biology
Benjamin Koenig, Yuchen Hao, Sophia Schwartz, Andrew J. R. Plested, Tobias Stauber
Article
Neurosciences
Hailan He, Xiaoshuang Cao, Fei Yin, Tenghui Wu, Tobias Stauber, Jing Peng
Summary: Vesicular chloride/proton exchangers of the CLC family are crucial for the endosomal-lysosomal pathway, and dysfunction may lead to severe disorders. A study reported a novel variant of CLCN6 associated with West syndrome, providing clinical and functional evidence for the link. The research shed light on the involvement of autophagic-lysosomal dysfunction in the pathogenesis of West syndrome.
MOLECULAR NEUROBIOLOGY
(2021)
Review
Cell Biology
Shroddha Bose, Hailan He, Tobias Stauber
Summary: This article discusses the role of CLC proteins in organellar ion homeostasis and neurodegenerative disorders, as well as the severe diseases associated with their abnormalities.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Clinical Neurology
Hailan He, Raul E. Guzman, Dezhi Cao, Juan Sierra-Marquez, Fei Yin, Christoph Fahlke, Jing Peng, Tobias Stauber
Summary: This study expanded the phenotypic and genetic spectrum of CLCN4-related epilepsy, showing that most patients have drug-resistant seizures starting in infancy, with cognitive and language impairment. Genotype-phenotype correlations were complex, with missense variants leading to more severe phenotypes.
Article
Endocrinology & Metabolism
Uta Roessler, Anna Floriane Hennig, Nina Stelzer, Shroddha Bose, Johannes Kopp, Kent Soe, Lukas Cyganek, Giovanni Zifarelli, Salaheddine Ali, Maja von der Hagen, Elisabeth Tamara Straessler, Gabriele Hahn, Michael Pusch, Tobias Stauber, Zsuzsanna Izsvak, Manfred Gossen, Harald Stachelscheid, Uwe Kornak
Summary: Human induced pluripotent stem cells (hiPSCs) have great potential for modeling human diseases and developing innovative therapeutic approaches. A novel differentiation method for forming functional osteoclasts from hiPSCs was reported, showing continuous production of monocyte-like cells for up to 9 weeks and generating sufficient material for multiple osteoclast differentiations. The study demonstrated that osteoclasts differentiated from hiPSCs could be used as a disease model for osteopetrosis and potentially other osteoclast-related diseases.
JOURNAL OF BONE AND MINERAL RESEARCH
(2021)
Editorial Material
Neurosciences
Hailan He, Tobias Stauber, Weiping Liao, Yuwu Jiang, Yongguo Yu, Jing Peng
FRONTIERS IN MOLECULAR NEUROSCIENCE
(2022)
Article
Endocrinology & Metabolism
Tobias Stauber, Lena Wartosch, Svenja Vishnolia, Ansgar Schulz, Uwe Kornak
Summary: After discovering a high level of v-ATPase complexes in the osteoclast ruffled membrane, it was found that the chloride/proton-exchanger ClC-7 could be responsible for transporting the negative charge. CLCN7 pathogenic variants were found in individuals with autosomal recessive osteopetrosis (ARO) and heterozygous pathogenic variants were identified as the exclusive cause of autosomal dominant osteopetrosis type 2 (ADO2). The electrophysiological properties of ClC-7 were also elucidated, showing that missense variants could lead to reduced chloride currents.
Article
Neurosciences
Hailan He, Huiwen Zhang, Hui Chen, Fang He, Fei Yin, Tobias Stauber, Xiaomin Zou, Jing Peng
Summary: The mutations in SLC9A6 gene lead to the loss of NHE6 protein, resulting in mitochondrial and lipid metabolism abnormalities, which play a role in the pathogenesis of Christianson syndrome. Importantly, filipin staining combined with electron microscopy examination can serve as a useful complementary diagnostic method for this syndrome.
CNS NEUROSCIENCE & THERAPEUTICS
(2023)
Article
Pharmacology & Pharmacy
Tianbao Liu, Yin Li, Dawei Wang, Tobias Stauber, Jiajun Zhao
Summary: In this study, bibliometric methods were used to assess the global scientific output and identify hotspots in research on the volume-regulated anion channel (VRAC) from 2014 to 2022. The analysis revealed that the United States was the leading country in this field, the University of Copenhagen was the most prominent institution, and Thomas J. Jentsch was the most published and cited author. Keyword co-occurrence analysis showed that VRAC is involved in various physiological processes, ion channels, and diseases.
FRONTIERS IN PHARMACOLOGY
(2023)
Review
Biochemistry & Molecular Biology
Lingye Chen, Fatemeh Hassani Nia, Tobias Stauber
Summary: This review focuses on the critical role of ion channels and transporters in developmental processes of muscle tissues, discussing their influence on myoblast differentiation, cardiac differentiation, and vascular smooth muscle cell differentiation. It highlights examples of model organisms or patients with mutations in ion channels, and proposes a potential underlying molecular mechanism involving hyperpolarization of the resting membrane potential and calcium signaling.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Biochemistry & Molecular Biology
Lingye Chen, Benjamin Koenig, Tobias Stauber
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS
(2020)