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Cells, Volume 11, Issue 17

September-1 2022 - 152 articles

Cover Story: MLC1-inherited mutations cause megalencephalic leukoencephalopathy with subcortical cysts (MLC), an incurable leukodystrophy characterized by macrocephaly, chronic brain edema, cysts and myelin vacuolation. Although MLC1 genetic defects affect VRAC functionality leading to ion/water exchange defects in astrocytes, MLC1’s proper function and MLC molecular pathogenesis are still unknown. We demonstrated that MLC1 is a CaMKII target protein whose phosphorylation, occurring in response to intracellular Ca2+ release, potentiates the VRAC-mediated activation of the swelling-induced chloride currents. These findings reveal that MLC1 is a Ca2+-regulated protein, linking volume control to Ca2+ signaling in astrocytes and provide new insight for the comprehension of the defective mechanisms underlining MLC and other brain pathologies characterized by astrocyte swelling and edema. View this paper
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Articles (152)

  • Article
  • Open Access
3 Citations
2,793 Views
14 Pages

Mineralocorticoid Receptor Antagonists Mitigate Mitral Regurgitation-Induced Myocardial Dysfunction

  • Wei-Ting Chang,
  • Yu-Wen Lin,
  • Chin-Yu Chen,
  • Zhih-Cherng Chen,
  • Jhih-Yuan Shih,
  • Chia-Ching Wu,
  • Chwan-Yau Luo and
  • Ping-Yen Liu

3 September 2022

Mitral regurgitation (MR), the disruption of the mitral valve, contributes to heart failure (HF). Under conditions of volume overload, excess mineralocorticoids promote cardiac fibrosis. The mineralocorticoid receptor antagonist spironolactone is a p...

  • Perspective
  • Open Access
5 Citations
3,087 Views
13 Pages

LACTB, a Metabolic Therapeutic Target in Clinical Cancer Application

  • Xiaohua Li,
  • Zhongkai Ren,
  • Xiaohong Huang and
  • Tengbo Yu

3 September 2022

Serine beta-lactamase-like protein (LACTB) is the only mammalian mitochondrial homolog evolved from penicillin-binding proteins and β-lactamases (PBP-βLs) in bacteria. LACTB, an active-site serine protease, polymerizes into stable filaments...

  • Article
  • Open Access
9 Citations
5,126 Views
18 Pages

Functional Intercellular Transmission of miHTT via Extracellular Vesicles: An In Vitro Proof-of-Mechanism Study

  • Roberto D. V. S. Morais,
  • Marina Sogorb-González,
  • Citlali Bar,
  • Nikki C. Timmer,
  • M. Leontien Van der Bent,
  • Morgane Wartel and
  • Astrid Vallès

3 September 2022

Huntington’s disease (HD) is a fatal neurodegenerative disorder caused by GAG expansion in exon 1 of the huntingtin (HTT) gene. AAV5-miHTT is an adeno-associated virus serotype 5-based vector expressing an engineered HTT-targeting microRNA (miH...

  • Communication
  • Open Access
8 Citations
4,805 Views
12 Pages

Experiences with Glofitamab Administration following CAR T Therapy in Patients with Relapsed Mantle Cell Lymphoma

  • Alexander D. Heini,
  • Ulrike Bacher,
  • Naomi Porret,
  • Gertrud Wiedemann,
  • Myriam Legros,
  • Denise Stalder Zeerleder,
  • Katja Seipel,
  • Urban Novak,
  • Michael Daskalakis and
  • Thomas Pabst

2 September 2022

Mantle cell lymphoma (MCL) is a rare type of B-cell Non-Hodgkin lymphoma (NHL) affecting predominantly male patients. While complete remissions following first-line treatment are frequent, most patients ultimately relapse, with a usually aggressive f...

  • Article
  • Open Access
6 Citations
2,879 Views
11 Pages

Reduced miR-146a-5p Is a Biomarker of Infant Respiratory Diseases Contributing to Immune Dysregulation in Small Airway Epithelial Cells

  • José M. Rodrigo-Muñoz,
  • Marta Gil-Martínez,
  • Clara Lorente-Sorolla,
  • Beatriz Sastre,
  • María Luz García-García,
  • Cristina Calvo,
  • Inmaculada Casas and
  • Victoria del Pozo

2 September 2022

Respiratory diseases such as bronchiolitis, and those with wheezing episodes, are highly important during infancy due to their potential chronicity. Immune response dysregulation is critical in perpetuating lung damage. Epigenetic modifications inclu...

  • Feature Paper
  • Article
  • Open Access
15 Citations
5,146 Views
17 Pages

ACTN2 Mutant Causes Proteopathy in Human iPSC-Derived Cardiomyocytes

  • Antonia T. L. Zech,
  • Maksymilian Prondzynski,
  • Sonia R. Singh,
  • Niels Pietsch,
  • Ellen Orthey,
  • Erda Alizoti,
  • Josefine Busch,
  • Alexandra Madsen,
  • Charlotta S. Behrens and
  • Moritz Meyer-Jens
  • + 15 authors

2 September 2022

Genetic variants in α-actinin-2 (ACTN2) are associated with several forms of (cardio)myopathy. We previously reported a heterozygous missense (c.740C>T) ACTN2 gene variant, associated with hypertrophic cardiomyopathy, and characterized by an...

  • Article
  • Open Access
14 Citations
4,807 Views
20 Pages

Impairment of Neuronal Mitochondrial Quality Control in Prion-Induced Neurodegeneration

  • Mo-Jong Kim,
  • Hee-Jun Kim,
  • Byungki Jang,
  • Hyun-Ji Kim,
  • Mohd Najib Mostafa,
  • Seok-Joo Park,
  • Yong-Sun Kim and
  • Eun-Kyoung Choi

2 September 2022

Mitochondrial dynamics continually maintain cell survival and bioenergetics through mitochondrial quality control processes (fission, fusion, and mitophagy). Aberrant mitochondrial quality control has been implicated in the pathogenic mechanism of va...

  • Article
  • Open Access
22 Citations
3,980 Views
22 Pages

Immune Determinants of Viral Clearance in Hospitalised COVID-19 Patients: Reduced Circulating Naïve CD4+ T Cell Counts Correspond with Delayed Viral Clearance

  • Mihaela Zlei,
  • Igor A. Sidorov,
  • Simone A. Joosten,
  • Mirjam H. M. Heemskerk,
  • Sebenzile K. Myeni,
  • Cilia R. Pothast,
  • Caroline S. de Brouwer,
  • A. Linda Boomaars-van der Zanden,
  • Krista E. van Meijgaarden and
  • Shessy T. Morales
  • + 18 authors

2 September 2022

Virus-specific cellular and humoral responses are major determinants for protection from critical illness after SARS-CoV-2 infection. However, the magnitude of the contribution of each of the components to viral clearance remains unclear. Here, we st...

  • Review
  • Open Access
19 Citations
3,649 Views
14 Pages

Comparative Analysis of Arsenic Transport and Tolerance Mechanisms: Evolution from Prokaryote to Higher Plants

  • Jie Zhang,
  • Jiayou Liu,
  • Fubin Zheng,
  • Min Yu,
  • Sergey Shabala and
  • Won-Yong Song

2 September 2022

Arsenic (As) is a toxic metalloid for all living organisms and can cause serious harm to humans. Arsenic is also toxic to plants. To alleviate As toxicity, all living organisms (from prokaryotes to higher plants) have evolved comprehensive mechanisms...

  • Article
  • Open Access
9 Citations
2,575 Views
13 Pages

Around-the-Clock Noise Induces AD-like Neuropathology by Disrupting Autophagy Flux Homeostasis

  • Pengfang Zheng,
  • Xiaojun She,
  • Chunping Wang,
  • Yingwen Zhu,
  • Bo Fu,
  • Kefeng Ma,
  • Honglian Yang,
  • Xiujie Gao,
  • Xiaofang Li and
  • Fangshan Wu
  • + 1 author

2 September 2022

Environmental noise is a common hazard in military operations. Military service members during long operations are often exposed to around-the-clock noise and suffer massive emotional and cognitive dysfunction related to an Alzheimer’s disease...

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Cells - ISSN 2073-4409