We characterized one of the identified variants (c.1208del; p.Asn403Ilefs*9), which induces the near absence of CCDC65 protein in patient sperm. In Chlamydomonas, CCDC65 ortholog (DRC2, FAP250) is a component of the Nexin-Dynein Regulatory complex (N-DRC), which interconnects microtubule ...
1. Rpn5 and Rpn11 are subunits of the lid subcomplex, Rpn2 of the base subcomplex, and α4 of the core particle subcomplex. B, whole-cell extracts of the indicated strains expressing proteasome subunits as GFP fusions from their chromosomal loci were prepared under non-denaturing conditions...
Focusing on this particular phenotype, we identified bi-allelic loss-of-function mutations in GAS8, a gene that encodes a subunit of the nexin-dynein regulatory complex (N-DRC) orthologous to DRC4 of the flagellated alga Chlamydomonas reinhardtii. Unlike the majority of PCD patients, individuals ...
Ciliary motility is regulated by the conserved nexin-dynein regulatory complex (N-DRC), which links adjacent doublet microtubules and regulates and coordinates the activity of outer doublet complexes. Despite its critical role in cilia motility, the assembly and molecular basis of the regulatory ...
The dynein regulatory complex (DRC) was first described in genetic screens for suppressor mutations that restore motility to paralyzed radial spoke (RS) or central pair (CP) mutants. The DRC is thought to identify intermediates in the signaling pathway between the CP, RS, and dynein arms. Cryo...
In situ localization of N and C termini of subunits of the flagellar nexin-dynein regulatory complex (N-DRC) using SNAP tag and cryo-electron tomography. J Biol Chem. 2015; 290 :5341–5353.Song K, Awata J, Tritschler D, Bower R, Witman GB, Porter ME, Nicastro D. In situ ...
The dynein regulatory complex is the nexin link and a major regulatory node in cilia and flagella. J Cell Biol 2009;187:921-933.Heuser T, Raytchev M, Krell J, Porter ME, Nicastro D. The dynein regulatory complex is the nexin link and a major regulatory node in cilia and flagella. J...
DRC3 is an assembly adapter of the nexin-dynein regulatory complex functional components during spermatogenesis in humans and miceGeneticsMolecular biologyReproductive disordersdoi:10.1038/s41392-022-01293-4Shushu Zhougrid.89957.3a0000 0000 9255 8984State Key Laboratory of Reproductive Medicine, Department ...
Song K, Awata J, Tritschler D, Bower R, Witman GB, Porter ME, Nicastro D. In situ localization of N and C termini of subunits of the flagellar nexin-dynein regulatory complex (N-DRC) using SNAP tag and cryo-electron tomography. J Biol Chem. 2015; 290 :5341–5353....