[HTML][HTML] Ciliogenesis membrane dynamics and organization

H Zhao, Z Khan, CJ Westlake - Seminars in cell & developmental biology, 2023 - Elsevier
Ciliogenesis is a complex multistep process used to describe assembly of cilia and flagella.
These organelles play essential roles in motility and signaling on the surface of cells. Cilia …

Composition, organization and mechanisms of the transition zone, a gate for the cilium

K Park, MR Leroux - EMBO reports, 2022 - embopress.org
The cilium evolved to provide the ancestral eukaryote with the ability to move and sense its
environment. Acquiring these functions required the compartmentalization of a dynein …

Reserpine maintains photoreceptor survival in retinal ciliopathy by resolving proteostasis imbalance and ciliogenesis defects

HY Chen, M Swaroop, S Papal, AK Mondal, HB Song… - elife, 2023 - elifesciences.org
Ciliopathies manifest from sensory abnormalities to syndromic disorders with multi-organ
pathologies, with retinal degeneration a highly penetrant phenotype. Photoreceptor cell …

Cellular and molecular mechanisms of pathogenesis underlying inherited retinal dystrophies

A Manley, BI Meshkat, MM Jablonski, TJ Hollingsworth - Biomolecules, 2023 - mdpi.com
Inherited retinal dystrophies (IRDs) are congenital retinal degenerative diseases that have
various inheritance patterns, including dominant, recessive, X-linked, and mitochondrial …

DCX-EMAP is a core organizer for the ultrastructure of Drosophila mechanosensory organelles

X Song, L Cui, M Wu, S Wang, Y Song, Z Liu… - Journal of Cell …, 2023 - rupress.org
Mechanoreceptor cells develop specialized mechanosensory organelles (MOs), where force-
sensitive channels and supporting structures are organized in an orderly manner to detect …

Drosophila transition fibers are essential for IFT-dependent ciliary elongation but not basal body docking and ciliary budding

Y Hou, S Zheng, Z Wu, C Augière, V Morel, E Cortier… - Current Biology, 2023 - cell.com
Cilia are highly conserved organelles critical for animal development and perception.
Dysfunction of cilia has been linked to a wide spectrum of human genetic diseases, termed …

Eupatilin improves cilia defects in human CEP290 ciliopathy models

JC Corral-Serrano, PE Sladen, D Ottaviani, OF Rezek… - Cells, 2023 - mdpi.com
The photoreceptor outer segment is a highly specialized primary cilium that is essential for
phototransduction and vision. Biallelic pathogenic variants in the cilia-associated gene …

[HTML][HTML] Pluripotent stem cell-derived models of retinal disease: Elucidating pathogenesis, evaluating novel treatments, and estimating toxicity

M Kurzawa-Akanbi, N Tzoumas… - Progress in Retinal and …, 2024 - Elsevier
Blindness poses a growing global challenge, with approximately 26% of cases attributed to
degenerative retinal diseases. While gene therapy, optogenetic tools, photosensitive …

Cep131-Cep162 and Cby-Fam92 complexes cooperatively maintain Cep290 at the basal body and contribute to ciliogenesis initiation

Z Wu, H Chen, Y Zhang, Y Wang, Q Wang… - Plos …, 2024 - journals.plos.org
Cilia play critical roles in cell signal transduction and organ development. Defects in cilia
function result in a variety of genetic disorders. Cep290 is an evolutionarily conserved …

Structure and function of distal and subdistal appendages of the mother centriole

D Ma, F Wang, J Teng, N Huang… - Journal of Cell …, 2023 - journals.biologists.com
Centrosomes are composed of centrioles surrounded by pericentriolar material. The two
centrioles in G1 phase are distinguished by the localization of their appendages in the distal …