Results 101 to 110 of about 17,107 (226)

Primary cilia contribute to the aggressiveness of atypical teratoid/rhabdoid tumors

Cell Death and Disease, 2022
Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant brain tumor in infants that is characterized by loss of nuclear expression of SMARCB1 or SMARCA4 proteins. Recent studies show that AT/RTs comprise three molecular subgroups, namely AT/RT-TYR,
Lena Blümel, Nan Qin, Johannes Berlandi, Eunice Paisana, Rita Cascão, Carlos Custódia, David Pauck, Daniel Picard, Maike Langini, Kai Stühler, Frauke-Dorothee Meyer, Sarah Göbbels, Bastian Malzkorn, Max C. Liebau, João T. Barata, Astrid Jeibmann, Kornelius Kerl, Serap Erkek, Marcel Kool, Stefan M. Pfister, Pascal D. Johann, Michael C. Frühwald, Arndt Borkhardt, Guido Reifenberger, Claudia C. Faria, Ute Fischer, Martin Hasselblatt, Jasmin Bartl, Marc Remke   +28 more
doaj   +1 more source

Insights into centriole geometry revealed by cryotomography of doublet and triplet centrioles. [PDF]

, 2018
Centrioles are cylindrical assemblies comprised of 9 singlet, doublet, or triplet microtubules, essential for the formation of motile and sensory cilia.
Agard, David A, Greenan, Garrett A, Keszthelyi, Bettina, Vale, Ronald D   +3 more
core   +1 more source

Microtubule-severing enzymes: From cellular functions to molecular mechanism. [PDF]

, 2018
Microtubule-severing enzymes generate internal breaks in microtubules. They are conserved in eukaryotes from ciliates to mammals, and their function is important in diverse cellular processes ranging from cilia biogenesis to cell division, phototropism ...
Ahmad, Akhmanova, Akhmanova, Allison, Antonina Roll-Mecak, Aubin-Tam, Banks, Barton, Benz, Bichet, Burk, Burk, Carlier, Carlier, Carlier, Charafeddine, Cheung, Chi, Connell, Denton, DeSantis, DesMarais, Dominguez, Dong, Doyle, Dymek, Dymek, Díaz-Valencia, Eckert, Evans, Francis J. McNally, Ganem, Gates, Grode, Guizetti, Han, Hara, Hartman, Hartman, Hawkins, Hazan, Hu, Hu, Iwaya, Jiang, Jiang, Jiang, Johjima, Joly, Kasher, Keating, Lacroix, Lata, Lee, Leo, Lindeboom, Liu, Loughlin, Lundin, Lučič, Maillard, Mains, Mao, Matsuo, McNally, McNally, McNally, McNally, McNally, McNally, Minoura, Mishra-Gorur, Mitchison, Monroe, Moretti, Mukherjee, Murata, Nakamura, Nicklas, Nithianantham, Olivares, Park, Peng, Portis, Puchades, Qiang, Rao, Reid, Rezabkova, Ribbeck, Rogers, Roll-Mecak, Roll-Mecak, Roll-Mecak, Roll-Mecak, Sharma, Sharp, Shaw, Sherwood, Sonbuchner, Srayko, Stamatakis, Stewart, Stone, Tao, Taylor, Ti, Tirnauer, Tran, Trotta, Vale, Valenstein, Vemu, Vemu, Vietri, Walker, Wang, Waterman-Storer, Wen, White, Whitehead, Wightman, Yang, Yang, Yang, Yu, Zehr, Zhang, Zhang, Zhang, Zhao   +130 more
core   +2 more sources

Unveiling a New Link: Cholesterol Deficiency in Smith–Lemli–Opitz and Niemann–Pick C as a Driver of Ciliopathies

American Journal of Medical Genetics Part A, Volume 200, Issue 6, Page 1179-1191, June 2026.
ABSTRACT The ciliopathies are a group of genetic disorders caused by defective function of either the primary cilia (a large number) or the motile cilia (a much smaller number). These have been defined as diseases with mutations in genes encoding individual ciliary or cilia‐associated proteins.
Robert P. Erickson, Maria Teresa Fiorenza   +1 more
wiley   +1 more source

Ciliogenesis and the DNA damage response: A stressful relationship [PDF]

, 2016
Both inherited and sporadic mutations can give rise to a plethora of human diseases. Through myriad diverse cellular processes, sporadic mutations can arise through a failure to accurately replicate the genetic code or by inaccurate separation of ...
Collis, SJ, Johnson, CA
core   +3 more sources

Excitatory neurons and astrocytes‐specific dysregulation and aberrant interactions are vulnerable to FCDI as suggested by single‐cell spatial transcriptomics

Clinical and Translational Medicine, Volume 16, Issue 5, May 2026.
• Excitatory neurons and astrocytes show marked alterations in the single‐cell spatial transcriptomic atlas of epileptogenic cortex from patients with focal cortical dysplasia type I (FCDI). • The CBLN2high excitatory neuron subtype may be associated with neuronal hyperexcitability and cortical development in FCDI.
Yaqian Zhang, Qihang Zou, Yingying Liu, Yinchao Li, Yubao Fang, Tiancai Huang, Jiabin Yu, Lisen Sui, Dezhi Cao, Liemin Zhou   +9 more
wiley   +1 more source

Dynein-2 intermediate chains play crucial but distinct roles in primary cilia formation and function

eLife, 2018
The dynein-2 microtubule motor is the retrograde motor for intraflagellar transport. Mutations in dynein-2 components cause skeletal ciliopathies, notably Jeune syndrome. Dynein-2 contains a heterodimer of two non-identical intermediate chains, WDR34 and
Laura Vuolo, Nicola L Stevenson, Kate J Heesom, David J Stephens   +3 more
doaj   +1 more source

A pathway for Parkinson's Disease LRRK2 kinase to block primary cilia and Sonic hedgehog signaling in the brain [PDF]

, 2018
Parkinson’s disease-associated LRRK2 kinase phosphorylates multiple Rab GTPases, including Rab8A and Rab10. We show here that LRRK2 kinase interferes with primary cilia formation in cultured cells, human LRRK2 G2019S iPS cells and in the cortex of LRRK2 ...
Alessi, Dario R., Dhekne, Herschel S., Diez, Federico, Gomez, Rachel C., Pfeffer, Suzanne R., Schüle, Birgitt, Steger, Martin, Tonelli, Francesca, Yanatori, Izumi   +8 more
core   +2 more sources

Club Cells in Nasal Epithelial Repair: Insights From an IL‐13‐Induced Inflammatory Model

Allergy, Volume 81, Issue 5, Page 1459-1471, May 2026.
Four inflammatory endotypes of CRSwNP, defined by eosinophil and neutrophil infiltration, show distinct patterns of basal, club, ciliated, and goblet cell expression in nasal epithelium. Using an ALI‐HNECs model, we demonstrate that IL‐13 impairs mucociliary function by promoting club‐to‐goblet cell differentiation while inhibiting club‐to‐ciliated ...
Zhi‐Qun Huang, Jing Liu, Jing Ye, Li‐Ying Sun, Hsiao Hui Ong, Yang Peng, Bing‐Qi Lin, Jun‐Hao Tu, Ke‐Shuang Wang, Yi‐Shan Xiong, Qing Luo, Mark Thong, Yu Xu, De‐Yun Wang   +13 more
wiley   +1 more source

A homozygous genome‐edited Sept2‐EGFP fibroblast cell line [PDF]

, 2019
Septins are a conserved, essential family of GTPases that interact with actin, microtubules, and membranes and form scaffolds and diffusion barriers in cells. Several of the 13 known mammalian septins assemble into nonpolar, multimeric complexes that can
Banko, Monika, Ewers, Helge, Heyd, Florian, Mucha‐Kruczynska, Iwona, Weise, Christoph   +4 more
core   +1 more source