Results 31 to 40 of about 47,507 (264)

FANCONI SYNDROME SECONDARY TO CYSTINOSIS IN YOUNG MALE: A CASE REPORT

Khyber Medical University Journal, 2021
INTRODUCTION:  Nephropathic cystinosis is a rarely occurring inherited metabolic disorder, leading to Fanconi syndrome, progressive renal failure and a range of extra-renal manifestations including endocrinopathies.
Samina Bibi, Mian Sameer Ahmed, Bakht Babar, Tahir Ghaffar, Azizul Hassan Aamir, Sana Aziz   +5 more
doaj   +1 more source

Selective elimination of quiescent cancer cells by dequalinium chloride via ROS‐mediated ferroptosis

FEBS Open Bio, EarlyView.
Dequalinium chloride (DQ) selectively eliminates quiescent cancer cells (QCCs) by amplifying mitochondrial ROS. QCCs exhibit elevated basal ROS and limited redox‐buffering capacity, making them vulnerable to further ROS increases. DQ‐induced ROS exceeds the tolerance threshold, triggering lipid peroxidation and ferroptosis, whereas proliferating cells ...
Kotaro Miyamoto, Shiori Sakai, Minori Endo, Terigele, Mizuho Niibori, Taiki Morita, Yu Goto, Arisa Yoshida, Tomohiro Tanaka, Ning Lin, Takahiro Kuchimaru, Hiroyuki Nakamura, Masahiro Inoue, Shinae Kizaka‐Kondoh, Tetsuya Kadonosono   +14 more
wiley   +1 more source

The Role of Cystinosin in the Intermediary Thiol Metabolism and Redox Homeostasis in Kidney Proximal Tubular Cells

Antioxidants, 2018
Cystinosin is a lysosomal transmembrane protein which facilitates transport of the disulphide amino acid cystine (CySS) from the lysosomes of the cell.
Rodolfo Sumayao, Philip Newsholme, Tara McMorrow   +2 more
doaj   +1 more source

Upcycling Keratin‐Based Textile Waste Into High‐Performance Hybrid Fibers Via Tuning the Interfacial Interactions

Advanced Functional Materials, EarlyView.
Keratin recovered from animal‐hair textile waste is fractionated and blended with cellulose to produce continuously spun hybrid fibers. Selecting the high‐molecular‐weight keratin fraction enables 30 wt.% keratin incorporation while maintaining high strength in dry and wet states.
Mian Zhai, Daniel Van Opdenbosch, Inge Schlapp‐Hackl, Teemu Välisalmi, Markus Linder, Michael Hummel, Wenwen Fang   +6 more
wiley   +1 more source

Cystinuria in an Australian Cattle Dog Family—A Seemingly Androgen-Associated Autosomal Dominant Trait

Veterinary Sciences
In Australian Cattle Dogs (AUCDs), cystinuria was reported to be an autosomal dominant trait caused by a 6 bp deletion in the SLC3A1 gene (type II-A). Here we report an androgen association in this breed.
Alexandra Kehl, Maria Brockmann, Sabine Helmes, Andrea Hildebrand, Sabine Döll, Elisabeth Mueller, Urs Giger   +6 more
doaj   +1 more source

Environmental cystine drives glutamine anaplerosis and sensitizes cancer cells to glutaminase inhibition

eLife, 2017
Many mammalian cancer cell lines depend on glutamine as a major tri-carboxylic acid (TCA) cycle anaplerotic substrate to support proliferation. However, some cell lines that depend on glutamine anaplerosis in culture rely less on glutamine catabolism to ...
Alexander Muir, Laura V Danai, Dan Y Gui, Chiara Y Waingarten, Caroline A Lewis, Matthew G Vander Heiden   +5 more
doaj   +1 more source

Covalent Functionalization of 2D Semiconductors: A Roadmap to Advanced Electronic Devices

Advanced Functional Materials, EarlyView.
This Review presents recent advances in the covalent functionalization strategies for two‐dimensional semiconductors and their implementation in modern technologies. Layered materials are modified through diverse molecular chemistries (e.g., thiols, diazonium salts, alkyl halides, and electron‐deficient species) to tailor their surface properties ...
Ramiro Quirós‐Ovies, Bin Han, Paolo Samorì   +2 more
wiley   +1 more source

Disulfidptosis contributes to rotenone-induced dopaminergic neuron damage

Neural Regeneration Research
Parkinson’s disease is a neurodegenerative disorder whose pathogenesis remains incompletely understood. Rotenone exposure is reportedly associated with Parkinson’s disease.
Wenqi Ye, Qifu Zhang, Wei Ge, Haoyin Liu, Yaohui Shan, Feng Ye, Xiaogang Wang, Yuanpeng Zhao, Guorong Dan, Mingliang Chen, Yan Sai   +10 more
doaj   +1 more source

DETOXICATION OF CYANIDE BY CYSTINE

Journal of Biological Chemistry, 1956
SUMMARY Chemical studies on the nature of the reaction product between cystine and cyanide ion support formulation of the structure as 2-imino-4-thiazoli- dinecarboxylic acid. The compound was inert metabolically when fed to the rat or when injected. On treatment with acid, a small amount of thio- cyanate was produced.
J L, WOOD, S L, COOLEY
openaire   +2 more sources

GHRHR Deficiency Enhances Retinal Ganglion Cell Survival and Visual Functions in Experimental Glaucoma by Inhibiting Ferroptosis

Advanced Science, EarlyView.
Glaucoma, a major cause of blindness, involves retinal ganglion cell (RGC) degeneration. This study shows growth hormone‐releasing hormone receptor (GHRHR) deficiency preserves RGC survival and restores vision, unlike activation which only aids survival.
Yan Tong, Ming Ho Yam, Jiaxin Zhang, Lin Du, Linbin Zhou, Yolanda Wong Ying Yip, Bo Man Ho, Hemlata Bisnauthsing, Jiahui Li, Ivan Kong, Shushu Xu, Changzhen Fu, Karl K.H. So, Joaquim S.L. Vong, Ling‐Ping Cen, Ming‐Ming Yang, Khazeema Yousaf, Mai Har Sham, Sun On Chan, Poemen P. Chan, Chi Pui Pang, Clement C. Tham, Jing Na He, Jian Li, Wai Kit Chu   +24 more
wiley   +1 more source