Results 81 to 90 of about 112,923 (261)

Gray platelet syndrome as a multisystem disorder: immune dysregulation, autoimmunity, and metabolic abnormalities

Annals of Hematology
Gray platelet syndrome (GPS) is a rare inherited platelet disorder caused by defective α-granule biogenesis. Emerging evidence suggests that GPS represents a multisystem disorder with immune and inflammatory manifestations.
Hussam Alzeerelhouseini, Leen Aljunaidi, Mays Alhouseini, Hani Saleh   +3 more
doaj   +1 more source

The IRE1‐XBP1s Axis Drives Inflammatory Osteolysis by Regulating a 5‐HT Dependent Endogenous Anti‐Autophagy Mechanism

Advanced Science, EarlyView.
A previously unrecognized IRE1‐XBP1s‐Slc6a4 signaling axis links endoplasmic reticulum stress to serotonin metabolism, autophagy, and inflammatory osteoclastogenesis. By promoting intracellular serotonin uptake and reducing endogenous 3‐methyladenine accumulation, this pathway accelerates inflammatory bone destruction and provides a promising ...
Pengchao Yang, Binxiang Zhu, Yuzhi He, Yang Tian, Honglei Kang, Shian Hu, Pengju Wang, Yong Xu, Zhuowei Lei, Peijun Qi, Hao Yang, Yang Lin, Yimin Dong, Feng Li, Hanfeng Guan   +14 more
wiley   +1 more source

Natural history study of patients with familial platelet disorder with associated myeloid malignancy. [PDF]

Blood, 2023
Cunningham L, Merguerian M, Calvo KR, Davis J, Deuitch NT, Dulau-Florea A, Patel N, Yu K, Sacco K, Bhattacharya S, Passi M, Ozkaya N, De Leon S, Chong S, Craft K, Diemer J, Bresciani E, O'Brien K, Andrews EJ, Park N, Hathaway L, Cowen EW, Heller T, Ryan K, Barochia A, Nghiem K, Niemela J, Rosenzweig S, Young DJ, Frischmeyer-Guerrerio PA, Braylan R, Liu PP.   +31 more
europepmc   +1 more source

Modulating Calcium Homeostasis via a Biomimetic Scaffold to Rescue Diabetic Ischemic Wounds

Advanced Science, EarlyView.
This strategy addresses impaired microcirculation and loss of extracellular matrix (ECM) guidance in diabetic wound healing. Musc@CP, a nanofibrous dressing combining an ECM‐mimetic chitosan‐pullulan scaffold with muscone, enhances perfusion by attenuating intracellular Ca2+ overload‐associated endothelial dysfunction.
Xiang Zheng, Mingmei Li, Jianjun Jiang, Yongchao Wang, Yaru Jia, Weilun Sun, Yi Yao, Pengli Gao, Linhua Zhang, Qi Guo, Guang Jia, Xing‐Jie Liang, Dunwan Zhu, Jinchao Zhang, Fangzhou Li   +14 more
wiley   +1 more source

Dynamic Interfacial pH Stabilization and (002) Oriented Deposition Enabled by Histidine‐Induced Solid Electrolyte Interphase for Highly Reversible Zn Anodes

Advanced Science, EarlyView.
The histidine‐induced ultrathin SEI (Zn(OH)2‐HIS) delivers exceptional anode stability by stabilizing the interfacial pH via a synergistic mechanism of chemical buffering and physical blocking, while inducing dendrite‐free oriented (002) deposition.
Qi Liu, Yimin Chen, Jianwei Lu, Xiangqun Zhuge, Xiyuan Zhong, Huaichong Sun, Yibing Li, Zhihong Luo, Kun Luo, Weiwei Lei, Dan Liu, Anjun Hu, Aijing Ma   +12 more
wiley   +1 more source

Clinical and laboratorial characterization of a cohort of patients with hereditary platelet disorders in Brazil

Hematology, Transfusion and Cell Therapy
Introduction: Inherited platelet disorders are rare conditions characterized by altered platelet function and/or reduced platelet counts. Diagnosing these disorders is challenging and may result in delays, misdiagnosis, and inappropriate treatment.
Letícia Dalla Vecchia Grassi, Erica Okazaki, Cynthia Rothschild, Paula Villaça, Fernanda Andrade Orsi, Bianca Stefanello   +5 more
doaj   +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

A splice mutation in RASGRP2 gene in the patient with recurrent epistaxis and nasal vascular malformation

Platelets
Platelet type bleeding disorder-18 (BDPLT18) caused by mutations of Ras guanyl releasing protein 2 (RASGRP2) is a relatively rare, new autosomal recessive disorder.
Zhong-Yu Shi, Qing-Ling Lei, Shao-Qin Duan, Yan Zhou, Ting-Ting Cui, Yun-Bi Lin, Chun-Hui Yang, Chun-Yan Song, Chun-Lian Fang, Xin Tian, Xian-Wen Zhang, Ti-Long Huang   +11 more
doaj   +1 more source

A Personalized Haplotype‐Resolved Near‐Gapless Genome Framework for Somatic Variant Discovery in Hepatocellular Carcinoma

Advanced Science, EarlyView.
A patient‐specific, haplotype‐resolved genome framework improves detection and interpretation of somatic variants in hepatocellular carcinoma. Using multi‐platform sequencing, the personalized assembly resolves complex regions including centromeres and MHC/HLA loci, enhances structural variant discovery, and links regulatory alterations to allele ...
Jiazheng Lin, Yao Xiao, Shuangzi Luo, Yifan Li, Wenfei Zhang, Xiaoyu Zhang, Xinyi Wang, Di Wu, Jieru Hong, Yongkun Luan, Xun Huang, Yixiu Wang, Ningbo Wu, Xiaoxiao Hu, Fei Zhao, Wei Li, Xiao Luo, Bi Shi   +17 more
wiley   +1 more source

Reorganization of Innate Immune Cell Lipid Profiles by Bioinspired Meroterpenoids to Limit Inflammation

Advanced Science, EarlyView.
Resolution pharmacology is an emerging strategy to tackle inflammatory pathologies. Bioinspired meroterpenoids induce a lipid mediator class switch toward inflammation resolution in vitro and in vivo by targeting key nodes in lipid mediator biosynthesis and neutral lipid dynamics.
Lorenz Waltl, David Holubek, Klaus Speck, Raphael Wildermuth, Franz‐Lucas Haut, Stephan Permann, Immanuel Plangger, Christian Steinborn, Zhigang Rao, Danilo D'Avino, Ida Cerqua, Julia Stadler, Fiorentina Roviezzo, Peter Schlenke, Anita Siller, Harald Schennach, Solveigh C. Koeberle, Eva‐Maria Pferschy‐Wenzig, Antonietta Rossi, Thomas Magauer, Andreas Koeberle   +20 more
wiley   +1 more source