Results 61 to 70 of about 36,737 (275)

NARFL Knockout Triggers Ferroptosis‐Driven Vascular Endothelial Dysfunction

Advanced Science, EarlyView.
NARFL is vital for CIA and oxidative stress resistance. NARFL deletion in HPMEC cells, zebrafish, and mice is lethal and rescued by a Ferroptosis inhibitor. NARFL deficiency disrupted its interaction with CIA proteins, decreased aconitase activity, increased IRP1 activity, induced Fe overload, and led to ferroptosis and oxidative stress, resulting in ...
Hui Hu, Jing Luo, Li Yu, Daoxi Qi, Boyu Li, Yating Chen, Chen Wang, Xiaokang Zhang, Wenzheng Guo, Qiyong Lou, Gang Zhai, Yonglin Ruan, Jianfei Huang, Shengchi Shi, Zhan Yin, Fang Zheng   +15 more
wiley   +1 more source

Rationale, application and clinical qualification for NT-proBNP as a surrogate end point in pivotal clinical trials in patients with AL amyloidosis [PDF]

, 2016
Amyloid light-chain (LC) amyloidosis (AL amyloidosis) is a rare and fatal disease for which there are no approved therapies. In patients with AL amyloidosis, LC aggregates progressively accumulate in organs, resulting in organ failure that is ...
Ando, Y, Comenzo, RL, Dispenzieri, A, Gertz, MA, Grogan, M, Lousada, I, Maurer, MS, Merlini, G, Palladini, G, Sanchorawala, V, Wechalekar, A   +10 more
core   +1 more source

Allosteric Modulation of Pathological Ataxin‐3 Aggregation: A Path to Spinocerebellar Ataxia Type‐3 Therapies

Advanced Science, EarlyView.
This study uncovers a new allosteric site in the Josephin domain of ataxin‐3 targeted by the molecular tweezer CLR01, which modulates protein aggregation, improves synaptic function in neuronal cells, and delays motor dysfunction in animal models.
Alexandra Silva, Sara Duarte‐Silva, Pedro M. Martins, Beatriz Rodrigues, Débora Serrenho, Daniela Vilasboas‐Campos, Andreia Teixeira‐Castro, Julio Vieyto‐Nuñez, Joel Mieres‐Perez, Francisco Figueiredo, Martyna Podlasiak, Tatiana Van‐der‐Kellen, Joana Fraga, James Noble, Carter Lantz, Niki Sepanj, Daniela Monteiro‐Fernandes, Sara Guerreiro, Andreia Neves‐Carvalho, Joana Pereira‐Sousa, Frank‐Gerrit Klärner, Thomas Schrader, Joseph A. Loo, Annalisa Pastore, Elsa Sanchez‐Garcia, Gal Bitan, Ana Luísa Carvalho, Patrícia Maciel, Sandra Macedo‐Ribeiro   +28 more
wiley   +1 more source

Extensive amyloid deposits in bone marrow aspirate smears

Clinical Case Reports, 2020
Amyloid light‐chain (AL) amyloidosis is the most common form of systemic amyloidosis. It can cause progressive organ dysfunction and eventually death, mainly due to cardiac involvement. Amyloidosis may rarely present as extensive amorphous, purplish‐blue
Ting Hon Stanford Li, Kit Fai Wong, Wai Shan Wong   +2 more
doaj   +1 more source

Skeletal Myopathy as the Initial Manifestation of Light Chain Multiple Myeloma

European Journal of Case Reports in Internal Medicine, 2020
Monoclonal gammopathies due to plasma cell dyscrasias can cause various rare neuromuscular disorders. The peripheral nervous system is most commonly affected, while muscle diseases associated with monoclonal gammopathies are rare. Skeletal myopathy, as a
Mohamed Reda Belkhribchia, Sara Moukhlis, Tarik Bentaoune, Najat Chourkani, Mohamed Zaidani, Mehdi Karkouri   +5 more
doaj   +1 more source

Crystal structure of monomeric human β-2- microglobulin reveals clues to its amyloidogenic properties [PDF]

, 2002
Dissociation of human β-2-microglobulin (β(2)m) from the heavy chain of the class I HLA complex is a critical first step in the formation of amyloid fibrils from this protein.
A. P. Kalverda, Adams, Becker, Bellotti, Berman, Booth, C. H. Trinh, Collaborative Computational Project Number, Connors, D. P. Smith, Delaglio, Esposito, Floege, Hoshino, Kabsch, Kad, Khan, Khurana, Laskowski, Linke, McParland, Merritt, Morgan, Ohhashi, Okon, Richardson, S. E. Radford, S. E. V. Phillips, Saper, Smith, Verdone, Wang   +31 more
core   +2 more sources

Long‐Term Outcomes of Reduced Intensity Conditioning Hematopoietic Stem Cell Transplantation for Systemic Sclerosis Patients with Impaired Cardiac Function

Arthritis &Rheumatology, Accepted Article.
Objective High intensity conditioning autologous hematopoietic stem cell transplantation (AHSCT) is standard of care for patients with advanced SSc. The role of reduced intensity conditioning (RIC) prior to AHSCT in this population remains unclear. We conducted this study to determine the long‐term outcomes of RIC AHSCT in SSc patients with cardiac ...
Yonatan Lean, Carrie Richardson, Anthony Esposito, Katy Bedjeti, George E. Georges   +4 more
wiley   +1 more source

Localised Laryngeal Amyloidosis Endoscopic Excision -A Case Report [PDF]

, 2013
:We report a case of primary laryngeal amyloidosis in a 35 year old adult patient who presented with hoarseness of voice for 6 months duration with no other symptoms.
Sundhar Krishnan, Gurunathan, Varma, Srinivasa, Vikram, Venakata Subramanian Jagannathan   +2 more
core   +1 more source

Causal Effects of Hydrophilic Bile Acids on Carfilzomib‐Related Cardiovascular Events in Multiple Myeloma: A Mendelian Randomization Study

Clinical Pharmacology &Therapeutics, EarlyView.
Carfilzomib is highly effective in the treatment of multiple myeloma, but it has been associated with cardiovascular adverse events that impact patient outcomes. Our prior global metabolomic analyses indicated an association between hydrophilic bile acids and carfilzomib‐cardiotoxicity risk, although a causal relationship remained to be determined ...
Samia Shabnaz, Eric Farber‐Eger, Marwa Tantawy, Neyousha Shahisavandi, Timothy J. Garrett, Samuel M. Rubinstein, Michael G. Fradley, Mohammed E. Alomar, Danny DeAvila, Kenneth H Shain, R. Frank Cornell, Daniel Lenihan, Qing Lu, Quinn S Wells, Rachid C. Baz, Yan Gong   +15 more
wiley   +1 more source

The clinical characteristics of immunoglobulin light chain amyloidosis in Chinese population: A systematic scoping review [PDF]

, 2021
Chengcheng Fu, Xiaohong Wang, Bin Wang, Lingjie Xu, Wen-Ming Chen   +4 more
openalex   +1 more source