Results 131 to 140 of about 591,453 (305)

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

Beyond Inborn Errors of Immunity: Brazilian Newborn Screening with KRECs Enabling Early Diagnosis of Hematologic Disorders

Journal of Human Immunity
BackgroundNewborn screening (NBS) with kappa-deleting recombination excision circles (KRECs) is primarily used to detect B cell lymphopenia and agammaglobulinemia as markers of inborn errors of immunity (IEIs).
Paola Suhet, Mariana Gouveia-Pereira Pimentel, Carolina S. Aranda, Jolan E. Walter, Antônio Condino-Neto   +4 more
doaj   +1 more source

Pegademase bovine (PEG-ADA) for the treatment of infants and children with severe combined immunodeficiency (SCID) [PDF]

, 2009
Adenosine deaminase deficiency (ADA) is a rare, inherited disorder of purine metabolism characterized by immunodeficiency, failure to thrive and metabolic abnormalities. A lack of the enzyme ADA allows accumulation of toxic metabolites causing defects of
Claire Booth, Booth, C, H Bobby Gaspar, Gaspar, HB   +3 more
core  

Integrated Single‐Cell and TCR Profiling Reveals Protection‐Associated CD8+ T Cell Subsets Linked to Viral Control in PRRSV

Advanced Science, EarlyView.
PRRSV vaccine‐mediated protection is associated with clonally expanded cytotoxic CD8+ T cell subsets driven by viral structural proteins and supported by innate TLR4/TLR8 signaling and CD4+ T cell help, whereas non‐protective responses are characterized by dysfunctional exhausted‐like CD8+ T cells linked to poor viral control.
Can Kong, Siang Chen, Maolin Li, Meng Wang, Hailin Zhang, Bolun Zhou, Zhenhua Xie, Chen Wang, Peng Gao, Jianjun Luo, Hanchun Yang, Runsheng Chen, Dongdong Zhang, Jie Li, Jun Han   +14 more
wiley   +1 more source

Mutations in the _SC4MOL_ gene encoding a novel methyl sterol oxidase cause autosomal recessive psoriasisiform dermatitis, microcephaly and developmental delay [PDF]

, 2008
Disorders of cholesterol biosynthesis have clinical manifestations involving skeleton, eyes, neurologic development, and skin. We describe a patient with congenital cataracts, developmental delay, microcephaly, and low serum cholesterol who developed ...
Jushua Michel, Miao He, Jacqueline Hoover, Lisa Kratz, Laura Ferris, Gerard Vockley, Richard Kelley, Michael Gibson, Abbe Vallejo   +8 more
core  

BCG HSP70 Reprograms Macrophages via Central Trained Immunity to Suppress Prostate Cancer

Advanced Science, EarlyView.
BCG‐derived HSP70 (Dnak) safely induces central trained immunity via epigenetic, metabolic, and O‐GlcNAcylation reprogramming of bone marrow progenitors, driving tumor‐associated macrophages toward an M1‐like phenotype to suppress prostate cancer. ABSTRACT Trained immunity offers a promising yet clinically challenging strategy for cancer immunotherapy,
Peng Liu, Kangkang Liu, Keruo Wang, Heyang Guan, Jiaming Fan, Taipeng Li, Jiaru Hao, Zeyuan Wang, Yongchao Yan, Jiatong Chen, Yuan Ma, Yang Yang, Yuanjie Niu   +12 more
wiley   +1 more source

A Rare De Novo Missense Mutation in IFT122 Confers a Genetic Susceptibility Factor of Idiopathic Pediatric Uveitis Via Trio‐based Whole‐Exome Sequencing

Advanced Science, EarlyView.
A rare de novo IFT122‐A773E variant is identified in idiopathic pediatric uveitis and shown to exacerbate retinal inflammation and barrier dysfunction. Mechanistically, the variant enhances IFT43 interaction, elevates calcium signaling, and activates the MEK/ERK/FRA1 axis, revealing a previously unrecognized cilia‐associated pathway that may increase ...
Qian Zhou, Jiaxing Huang, Yilin Wang, Xingran Li, Xianyang Liu, Lingyu Dai, Hongshun Li, Qingfeng Wang, Jiangyi Liu, Guannan Su, Wanyun Zhang, Yang Deng, Qingfeng Cao, Yujie Lai, Xiang Luo, Changwei Huang, Ling Chen, Shengping Hou, Peizeng Yang   +18 more
wiley   +1 more source

Chimeric IL‐6/4R‐LL37 Engineered Macrophages Achieve Synchronized Inflammation Control and Antimicrobial Defence in Sepsis

Advanced Science, EarlyView.
Engineered macrophages programmed in situ by LNP‐delivered IL‐6/4 fusion and LL37 mRNAs simultaneously dampen cytokine storm, promote M2‐like repair, and enhance direct bacterial killing in sepsis. This combinatorial strategy restores T cell and macrophage function, lowers organ bacterial burden, and improves survival, highlighting a precision, host ...
Tianyang Jie, Zheyu Zhang, Hao Zhang, Linke Bian, Penglong Zheng, Sijia Zhou, Minqi Wang, Mingyu Qu, Yufei Wang, Xinhua Qu, Bing Yue   +10 more
wiley   +1 more source

Repurposing a Small Molecule Plant Hormone as a Tunable ON‐Switch for CAR‐T Cell Immunotherapy

Advanced Science, EarlyView.
By engineering a receptor system integrating the plant auxin receptor AFB1 with its co‐receptor IAA7, we enable ligand‐dependent interactions triggered by the plant hormone auxins. This design allows rapid, reversible, and dose‐dependent T cell activation, resulting in potent cytotoxicity against B‐cell lymphoma in vitro and in vivo.
Hongxiang Zeng, Lei Zheng, Rongkun Tao, Sirui Zheng, Yanke Zhong, Yongxia Niu, Peng Zhang, Xiang Ma, Chen Yang, Min Zhang, Fuyu Duan, Jing Yang, Xuan Wei, Xiang Zhou, Zhenbiao Yang, Zhaoxi Sun, Qizhou Lian   +16 more
wiley   +1 more source

Immunological Deficiency Syndromes [ Abridged ] [PDF]

Proceedings of the Royal Society of Medicine, 1968
  +14 more sources