Results 241 to 250 of about 2,946,422 (305)

Identifying a Csmd3+ Microglial Subpopulation that Drives Cold‐to‐Hot Transition and Immune‐Cure in Glioblastoma

Advanced Science, EarlyView.
The study establishes an immune‐cure (ICu) mouse model based on a TMEMed G422TN‐GBM system that faithfully recapitulates human TMEMed GBM. scRNA‐seq analysis reveals a Csmd3+ microglial subset with innate immune memory (IIM) potential that potently suppresses GBM growth, drives a TME cold‐to‐hot transition, and induces 100% ICu in long‐term survival ...
Hai‐Feng Jiang, Pan‐Pan Gao, Yu‐Wen Du, Li‐Qin Wu, En‐Zhi Yin, Qi An, Ze‐Hua Ding, Jin‐Wen Shi, Ya Shu, Ruoqiao Chen, Feng Liu, Mingfeng Li, Xiao Qian Chen   +12 more
wiley   +1 more source

Fetal and obstetrics manifestations of mitochondrial diseases. [PDF]

J Transl Med
Adelizzi A, Giri A, Di Donfrancesco A, Boito S, Prigione A, Bottani E, Bollati V, Tiranti V, Persico N, Brunetti D.   +9 more
europepmc   +1 more source

Cancer‐Associated BCL‐2 Mutants Reveal Mechanisms Towards Venetoclax Resistance

Advanced Science, EarlyView.
Venetoclax (VEN) resistance in chronic lymphocytic leukemia arises from diverse BCL2 mutations. We map mechanisms contributing to VEN resistance across common BCL‐2 variants. G101V and D103Y reduce drug binding and increase sequestration of pro‐apoptotic proteins. V156D blocks VEN allosterically.
Jonas Aufdermauer, Ian de Ridder, Mahjoobeh Ehsani, Marasim Khan, Justin Kale, Lukas P. Frenzel, David Andrews, Spyridoula Karamanou, Ana J. Garcia‐Saez, Geert Bultynck   +9 more
wiley   +1 more source

Endocrine Dysfunction in Primary Mitochondrial Diseases.

Endocr Rev
Varughese R, Rahman S.
europepmc   +1 more source

Social provisions in patients with mitochondrial diseases. [PDF]

BMJ Neurol Open
Haque S, Crawley K, Schofield D, Shrestha R, Davis R, Sue CM.   +5 more
europepmc   +1 more source

A Second Pathogenic Protein, PolyGN2C‐iso2, Reveals a Dual‐Protein Pathology in Neuronal Intranuclear Inclusion Disease

Advanced Science, EarlyView.
This study reveals that NOTCH2NLC transcript variant 2 generates PolyGN2C‐iso2, an aggregating protein present within intranuclear inclusions of NIID patient tissues. A novel mouse model expressing PolyGN2C‐iso2 recapitulates white matter abnormalities and cognitive deficits, mechanistically linked to mitochondrial dysfunction. These findings support a
Kang Zhang, Wenhao Ma, Yi Zhou, Zhijie Wu, Pan Gao, Hongze Niu, Hongfei Tai, Tianyi Zhao, Zheyue Dong, Li Li, Yan Zhang, An Wang, Si Shen, Yueyang Li, Sifei Yu, Yan Peng, Wang Sheng, Xiaoyan Dong, Hua Pan, Kaibin Shi, Magdalena J. Koziol, Xiaobing Wu, Zaiqiang Zhang   +22 more
wiley   +1 more source

Open-label pilot study using hydroxytyrosol as dietary supplements in patients with mitochondrial diseases. [PDF]

Orphanet J Rare Dis
Wong TS, Man E, Wong ICK, Li SX, Zhi H, Yeung KMC, Yip KY, Kwong AKY, Belaramani KM, Wong SN, Kwok AMK, Mak M, Chan TCH, Lau PY, Kan YLE, Wong KC, Poon F, Kwok V, Li CYD, Lau P, Lam SM, Chu V, Chan CFG, Fung CW.   +23 more
europepmc   +1 more source

Human Dental Pulp Stem Cell Secretome Restores Ischemic Stroke–Impaired Motor and Cognitive Functions by Reprogramming Redox and Inflammatory Signaling

Advanced Science, EarlyView.
Human dental pulp stem cell secretome emerges as a cell‐free therapeutic strategy for ischemic stroke by reprogramming redox and inflammatory signaling. hDPSC secretome‐derived antioxidant and immunomodulatory factors suppress the TLR4–NOX–ROS–NF‐κB axis, reduce microglial inflammatory responses and apoptosis, and promote neurogenesis, angiogenesis ...
Kyung‐Joo Seong, Sehoon Park, Sun‐Woong Bae, Daseul Kim, Jin Ho Lee, Won‐Seok Choi, Zee‐Yong Park, Ji‐Yeon Jung, Won‐Jae Kim   +8 more
wiley   +1 more source

The First Heterozygous <i>TWNK</i> Nonsense Mutation Associated with Progressive External Ophthalmoplegia: Evidence for a New Piece in the Puzzle of Mitochondrial Diseases. [PDF]

Biomolecules
Lopergolo D, Berti G, Gallus GN, Bianchi S, Santorelli FM, Malandrini A, De Stefano N.   +6 more
europepmc   +1 more source

A Blood‐Derived Factor Rescues ALS: Platelet Factor 4 Activates OPTN‐Dependent Autophagy to Clear SOD1 Aggregates Independently of PINK1

Advanced Science, EarlyView.
Systemic platelet factor 4 (PF4) is significantly depleted in amyotrophic lateral sclerosis (ALS). Peripheral PF4 replenishment restores central proteostasis by driving OPTN‐dependent, PINK1‐independent selective autophagy in motor neurons. This intervention effectively clears toxic SOD1 aggregates, blunts glial activation, and preserves neuromuscular ...
Qingjian Xie, Yanbo Zhu, Wenhua Jiang, Haobo Xie, Yaojia Li, Mengjie Hu, Kezheng Li, Huan Yu, Yixuan Pan, Chaoyi Jiang, Xueqin Song, Dongsheng Fan, Binbin Deng   +12 more
wiley   +1 more source