Results 181 to 190 of about 42,400 (289)

Amyloid‐β aggregates induce vasculopathy via ferroptosis in brain endothelial cells

Brain Pathology, EarlyView.
Blood–brain barrier (BBB) disruption is evident in brains with amyloidopathy. In this study, we demonstrate that amyloid β (Aβ) drives abnormal lipid metabolism and lipid droplet formation in brain endothelial cells, leading to lipid peroxidation and ferroptosis.
Suhyeon Son, Suji Kim, Min‐Tae Jeon, Moonseok Choi, Kyu‐Sung Kim, Suji Lee, Seung‐Yeol Nah, Do‐Geun Kim   +7 more
wiley   +1 more source

Parkinson's Disease: The Catabolic Theory [PDF]

, 2013
Manzoni, C
core  

TrkA abundance is increased in cutaneous nerves in bortezomib‐induced neuropathy

Brain Pathology, EarlyView.
Cutaneous nerves in bortezomib‐induced peripheral neuropathy (BIPN) show reduced nerve fiber density, increased TrkA expression, and enhanced dermal angiogenesis, highlighting a pathological switch in NGF/TrkA signaling that may contribute to nerve damage and pain. Abstract Tropomyosin receptor kinase A (TrkA), a high‐affinity receptor for nerve growth
Yuying Jin, Nadine Cebulla, Daniel Schirmer, Eva Runau, Leon Flamm, Calvin Terhorst, Laura Jähnel, Johanna Güse, Nicola Giordani, Annett Wieser, Julian Brennecke, Annemarie Sodmann, Martin Kortüm, Robert Blum, Claudia Sommer   +14 more
wiley   +1 more source

The DNA/RNA autophagy protein SIDT2 as a novel neuropathological hallmark in Huntington disease

Brain Pathology, EarlyView.
SIDT2‐immunoreactive inclusions are observed in the striatum, cerebral cortex, and hypothalamus in HD cases with different Vonsattel grades, and the frequency of SIDT2‐immunoreactive inclusions is associated with longer CAG repeats in the huntingtin gene.
Sanaz Gabery, Sofia Bergh, Chrisovalantou Huridou, Rachel Y. Cheong, Barbara Baldo, Paul Günther Scheunemann, Marie‐Louisa Schoebel, Linda Holmquist Mengelbier, Elisabet Englund, Catriona McLean, Carsten Saft, Deniz Kirik, Maria Björkqvist, Glenda Halliday, Elisabeth Petrasch‐Parwez, Huu Phuc Nguyen, Jonasz Jeremiasz Weber, Åsa Petersén   +17 more
wiley   +1 more source

Cannabidiol and cannabigerol ameliorate steatotic liver disease via phosphocreatine buffering and lysosomal restoration

British Journal of Pharmacology, EarlyView.
Cannabidiol (CBD) and cannabigerol (CBG) alleviate hepatic steatosis in obese mice by shifting energy buffering towards phosphocreatine and enhancing lysosomal lipid degradation and trafficking. These effects are associated with increased creatine kinase activity and lysobisphosphatidic acid (LBPA) levels, highlighting a previously unrecognised ...
Radka Kočvarová, Shahar Azar, Bella Agranovich, Ifat Abramovich, Saveliy Kirillov, Alina Nemirovski, Saja Baraghithy, Inbar Plaschkes, Emmanuelle Merquiol, Alexander Rouvinski, Galia Blum, Liad Hinden, Joseph Tam   +12 more
wiley   +1 more source

Relative acidic compartment volume as a lysosomal storage disorder-associated biomarker. [PDF]

J Clin Invest, 2014
te Vruchte D, Speak AO, Wallom KL, Al Eisa N, Smith DA, Hendriksz CJ, Simmons L, Lachmann RH, Cousins A, Hartung R, Mengel E, Runz H, Beck M, Amraoui Y, Imrie J, Jacklin E, Riddick K, Yanjanin NM, Wassif CA, Rolfs A, Rimmele F, Wright N, Taylor C, Ramaswami U, Cox TM, Hastings C, Jiang X, Sidhu R, Ory DS, Arias B, Jeyakumar M, Sillence DJ, Wraith JE, Porter FD, Cortina-Borja M, Platt FM.   +35 more
europepmc   +1 more source

Lysosomal storage disorders [PDF]

The Indian Journal of Pediatrics, 1999
openaire   +1 more source

The Molecular Diagnosis of Myopathies: Integrating Genomic, Proteomic, and Pathological Insights Toward Precision Medicine

Clinical Genetics, EarlyView.
Advances in genomic, proteomic, and transcriptomic technologies are transforming the diagnosis of genetic myopathies. When integrated with traditional muscle pathology, multi‐omics approaches improve diagnostic yield, clarify disease mechanisms, and support more precise, mechanism‐based therapeutic strategies for patients with neuromuscular disorders ...
Ludmila Alem, Denise de Abreu Pereira, Katia Carneiro   +2 more
wiley   +1 more source

SCD2 Alleviates Diabetes‐Associated Cognitive Dysfunction by Improving Microglial Lipid Metabolism

Cell Proliferation, EarlyView.
This study reveals a novel mechanism of microglial metabolic dysfunction in diabetic cognitive impairment. Defective SCD2 disrupts monounsaturated fatty acid (MUFA) metabolism, triggering mitochondrial oxidative phosphorylation dysfunction and leading to abnormal lipid droplet accumulation (marked by PLIN2).
Yang Yang, Juan He, Jing Zheng, Bing Shao, Lixin Shi, Miao Zhang   +5 more
wiley   +1 more source

A Rare Case of Cutaneous Crystal‐Storing Histiocytosis With Kappa Light Chain Restriction and Unusual BCL6 Expression

Journal of Cutaneous Pathology, EarlyView.
ABSTRACT Crystal‐storing histiocytosis (CSH) is a rare histopathologic phenomenon characterized by the accumulation of crystalline material within histiocytes, most often associated with lymphoplasmacytic or plasma cell neoplasms. Cutaneous involvement is uncommon and may present diagnostic challenges.
Liangli Wang, Jonathan L. Curry, Julia Dai, Daniel H. Wang, Paolo Strati, Melody R. Becnel, Leomar Y. Ballester, Carlos A. Torres‐Cabala, Phyu P. Aung   +8 more
wiley   +1 more source