Results 41 to 50 of about 3,017 (208)

Effect of co-culture with CTNS-expressing cells on cystine content of mutant CTNS cells.

, 2013
(a) Cystine content of CTNS(−/−) fibroblasts co-cultured with increasing amounts of wildtype amMSC. (b) Cystine content of GFP-tagged CTNS(−/−) ciPTEC isolated by FACS after co-culture with unlabelled wildtype amMSC, mutant ciPTEC or CTNS-corrected ...
Reyhan El-Kares (311952), Nicoletta Eliopoulos (311965), Jing Zhao (21160), Diana M. Iglesias (311951), Lambertus van den Heuvel (311962), Francesco Bellomo (311954), Jaan Toelen (311961), Yoon Kow Young (311964), LeeLee Chu (311963), Paul Goodyer (311966), Francesco Emma (311956), Anna Taranta (311953), Elena Levtchenko (311960), Martine Besouw (311958)   +13 more
core   +1 more source

CTNS Molecular Genetics Profile in a Portuguese Cystinosis Population [PDF]

, 2018
Background: Cystinosis is a multisystemic autosomal recessive deficiency of the lysosomal membrane transporter protein (cystinosin) caused by mutations in CTNS gene.
Costa, Teresa, Vilarinho, Laura, Sousa, David, Carmona, Célia, Ramos, Sónia, Neiva, Raquel, Ferreira, Filipa, Mota, Conceição, Carmo Macário, Maria do, Pinto, Helena, Magriço, Rita, Oliveira, João Paulo, Tavares, Isabel, Marcão, Ana, Leal, Inês, Lopes, Daniela, Gomes, Ana Marta   +16 more
core   +1 more source

Physics‐Based Compact Modeling of Advanced 3D Nanoscale Vertical NAND Flash Memory

Advanced Electronic Materials, EarlyView.
For advanced 3D NAND flash memory, a unified compact model for SPICE is proposed that spans from the intrinsic unit cell to the full string and captures the electrostatic coupling with adjacent inhibit strings. It can successfully predict read behavior, program/erase dynamics, and interactions between neighboring cells, reflecting array‐level behavior ...
Ilho Myeong, Seonho Shin, Ickhyun Song
wiley   +1 more source

Histological features of kidneys from Ctns^Y226X/Y226X vs. Ctns^+/+mice.

, 2019
A. Cystine crystals in 6-months old Ctns+/+ and CtnsY226X/Y226X female kidney. B. Bowman’s capsule/proximal tubule lesions in 6-month old CtnsY226X/Y226X male kidney vs Ctns+/+ wildtype.
Pedro Huertas (4891903), Emma J. Brasell (8081969), Lee Lee Chu (8081972), Yojiro Yamanaka (7589195), Iris Alroy (8081981), Paul Goodyer (311966), Brian M. Gilfix (8081987), Murielle M. Akpa (8081975), Idit Eshkar-Oren (8081978), Rachel Corsini (8081984)   +9 more
core   +1 more source

Phase II Study of Posttransplant Cyclophosphamide‐Based Graft‐Versus‐Host Disease Prophylaxis After HLA‐Mismatched Unrelated Donor Reduced Intensity Transplantation: Results From the ACCESS Trial Expansion Cohort

American Journal of Hematology, EarlyView.
ABSTRACT Posttransplant cyclophosphamide (PTCy) to prevent graft‐versus‐host disease (GVHD) improves outcomes in recipients of HLA mismatched unrelated donor (MMUD) allogeneic hematopoietic cell transplantation (allo HCT). Outcomes of MMUD HCT using PTCy in patients requiring reduced intensity or non‐myeloablative conditioning (RIC/NMA) are not well ...
Brian C. Shaffer, Monzr M. Al Malki, Antonio Martin Jimenez Jimenez, Joseph Stanek, Stephanie Bo‐Subait, Brent R. Logan, Jianqun Kou, Erin Leckrone, Heather E. Stefanski, Stephen R. Spellman, Craig Malmberg, Medhat Askar, Rachel Cusatis, Dipenkumar Modi, Farhad Khimani, Mehdi Hamadani, Martin Maiers, Rachel Cook, Karen Ballen, Javier Bolaños‐Meade, Uttam Rao, Jordan Milner, Ramzi Abboud, Katarzyna Jamieson, George Carrum, Bhagirathbhai Dholaria, William J. Hogan, Ran Reshef, Satyajit Kosuri, Alison Loren, Karilyn Larkin, Sally Arai, Muna Qayed, Sung Won Choi, Larisa Broglie, Jeffery J. Auletta, Bronwen E. Shaw, Steven M. Devine, Mahasweta Gooptu   +38 more
wiley   +1 more source

Compositional Time–Temperature–Transformation and Post‐Processing Window Mapping of Epoxy Nanocomposites via Machine Learning

Journal of Applied Polymer Science, EarlyView.
Discrete DSC and rheological data are transformed into continuous compositional Time–temperature–transformation (TTT) diagrams through physics‐informed Gaussian Process Regression. The proposed framework quantitatively predicts post‐gel processing windows across temperature and nanofiller composition, providing a practical route for accelerated design ...
Otávio Bianchi, Dante A. Benvenutti, Leonardo B. Canto, Alessandra Lavoratti, Zainab Al‐Maqdasi, Sandro Campos Amico, Patrik Fernberg   +6 more
wiley   +1 more source

Pang Yuk Cheung: Effects of oral cysteamine treatment on Ctns knockout rats

, 2022
Cystinosis is a rare genetic disorder caused by mutations in the gene CTNS which mainly affects the kidneys. Without treatment, this results in renal failure early in life.
Pang Yuk Cheung (1210017)
core   +1 more source

Leptin signalling altered in infantile nephropathic cystinosis‐related bone disorder

Journal of Cachexia, Sarcopenia and Muscle
Background The CTNS gene mutation causes infantile nephropathic cystinosis (INC). Patients with INC develop Fanconi syndrome and chronic kidney disease (CKD) with significant bone deformations.
Wai W. Cheung, Ping Zhou, Ronghao Zheng, Arieh Gertler, Eduardo A. Oliveira, Robert H. Mak   +5 more
doaj   +1 more source

An unusual titanosaur axis from the Upper Cretaceous of Brazil and its significance for sauropod anatomy and systematics

The Anatomical Record, EarlyView.
Abstract The Upper Cretaceous São José do Rio Preto Formation (Bauru Group, southeastern Brazil) has yielded a fragmentary but taxonomically diverse record of titanosaur sauropods, although elements from cervical series remain scarce. Here, we describe a nearly complete sauropod axis from the Vila Ventura Paleontological Area, representing an uncommon ...
Bruno A. Navarro, Jeffrey A. Wilson Mantilla, Jhonata H. A. Martins, Fabiano V. Iori, Leonardo S. Paschoa, Alberto B. Carvalho, Joseli M. Piranha, Hussam Zaher   +7 more
wiley   +1 more source

Impairment of chaperone‐mediated autophagy leads to selective lysosomal degradation defects in the lysosomal storage disease cystinosis

EMBO Molecular Medicine, 2015
Metabolite accumulation in lysosomal storage disorders (LSDs) results in impaired cell function and multi‐systemic disease. Although substrate reduction and lysosomal overload‐decreasing therapies can ameliorate disease progression, the significance of ...
Gennaro Napolitano, Jennifer L Johnson, Jing He, Celine J Rocca, Jlenia Monfregola, Kersi Pestonjamasp, Stephanie Cherqui, Sergio D Catz   +7 more
doaj   +1 more source