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The Ultrastructure of Olfactory and Nasal Respiratory Epithelium Surfaces
Published in D. V. M. Gerd Reznik, Sherman F. Stinson, Nasal Tumors in Animals and Man, 2017
Ciliogenesis apparently occurs for several cilia at the time, both in the case of olfactory (Figure 26) and respiratory cilia (Figure 27). Besides ciliary stubs, Figure 27 shows microvilli with high P-face particle densities. Necklace formation precedes formation of cilia (Figures 23, 25A, and 27).35,149,258 During outgrowth, and apparently also in later stages, olfactory cilia terminate club-shaped or vesicular (Figures 26 and 38 A).8,23,69,154
Management of congenitally corrected transposition from fetal diagnosis to adulthood
Published in Expert Review of Cardiovascular Therapy, 2023
ccTGA is a rare congenital heart disease (CHD) and accounts for approximately 0.03% of all CHDs. The annual incidence of ccTGA has been reported to be around 1/25,500 live births [8]. The etiology of the defect is not known. Recent studies indicate a relationship between the development of ccTGA and dysfunction of cilia. In a large multicenter study of 1043 unrelated ccTGA probands, an association between ccTGA, d-TGA, laterality defects, and in some cases primary ciliary dyskinesia was shown [9]. Another study revealed a high prevalence of an abnormal ciliary function in isolated ccTGA patients independent of laterality abnormalities [10]. Finally, mutations of genes involved in ciliogenesis and cilia-transduced signaling pathways in a ccTGA exome sequencing study have been described. An oligogenic or polygenic inheritance of the disease is suggested [11].
Meckel Gruber and Joubert Syndrome Diagnosed Prenatally: Allelism between the Two Ciliopathies, Complexities of Mutation Types and Digenic Inheritance
Published in Fetal and Pediatric Pathology, 2022
Somya Srivastava, Rani Manisha, Aradhana Dwivedi, Harshita Agarwal, Deepti Saxena, Vinita Agrawal, Kausik Mandal
Ciliopathies are a diverse group of disorders which occur due to dysfunction of motile or immotile cilia. They have 2 important functions-motility and cell signaling. Impaired ciliary motility exclusively results in primary ciliary dyskinesia which gives rise to disorders encompassing infertility, situs inversus, bronchiectasis and atelectasis. Impaired signaling and sensory function of motile or immotile cilia results in disorders of multiple organs (brain, eye, ear, kidney, liver, nose, skeleton). Ciliogenesis starts from a mother centriole which matures into a basal body, docks on the plasma membrane and further gives rise to the ciliary shaft. In between the shaft and the basal body lies the transition zone which controls the trafficking of molecules into and out of the cell [1].
PLK4: a link between centriole biogenesis and cancer
Published in Expert Opinion on Therapeutic Targets, 2018
Radhika Radha Maniswami, Seema Prashanth, Archana Venkataramana Karanth, Sindhu Koushik, Hemalatha Govindaraj, Ramesh Mullangi, Sriram Rajagopal, Sooriya Kumar Jegatheesan
Ciliogenesis begins with the nucleation of the mother centriole that functions as a basal body to template primary cilia [84]. PLK4-dependent phosphorylation of PCM1 is necessary for ciliogenesis. PLK4 phosphorylates PCM1 at a conserved S372 position and promotes dimerization/oligomerization. It also stimulates interaction of PCM1 with other centriolar satellite components such as hMsd1/SSX2IP, BBS4, and CEP290 resulting in increased aggregation and localization of centriolar satellites. Integrity of centriolar satellite components is an important prerequisite for ciliogenesis. It has been observed that depletion of PLK4 results in dispersion of centriolar satellites and perturbed ciliogenesis leading to loss of primary cilia [103]. This result in defective signaling since primary cilia coordinates a series of pathways that include Hedgehog, Wnt, PDGFRα, integrin and cell differentiation signaling pathways [104,105].