Variations in the ultrastructure of human nasal cilia including abnormalities found in retinitis pigmentosa.

Abstract

SUMMARYThe electron microscopic structure of cilia from the inferior turbinate of the nose was studied in 12 adults, four with chronic sinusitis, one with allergic rhinitis, two with bronchiectasis, three with deviated nasal septum, and two normals. The changes are compared with those found in nasal cilia in 14 patients with retinitis pigmentosa.There were compound cilia in the seven cases with chronic sinusitis, allergic rhinitis, and bronchiectasis but, apart from this, the structure of the cilia was similar in all 12 cases. There were variations in the microtubular pattern in about 4 % of cilia, dynein arms were not seen in 4 %, and in the rest an average of 5-6 dynein arms were seen in each cilium. The orientation of the cilia was 0 to 900.In the retinitis pigmentosa patients there was a highly significant increase in cilial abnormalities.The establishment on a quantitative basis of the variations in normal structure of nasal cilia facilitated the recognition of an association between cilial abnormalities and retinitis pigmentosa and should help in the identification of associations that may exist between cilial abnormalities and other diseases.In a recent study of ultrastructural abnormalities in nasal cilia in retinitis pigmentosa' we found it essential to establish in some quantitative form the type and range of abnormalities present in nasal cilia from a group of control subjects. This information will also be useful in the investigation of chronic lung diseases2-6 and sterility in men,7 8 in which there are known abnormalities in cilia, and should enable one to recognise associations of cilial abnormalities with other diseases.The electron microscopic structure of normal cilia and various abnormalities found are described, and quantitative measurements of the various changes are made and compared with those found in patients with retinitis pigmentosa. Subjects studied and methodsTwelve subjects (8 men, 4 women) were studied: two healthy volunteers, three patients with deviated

Structural heterogeneity of the axonemes of respiratory cilia and sperm flagella in normal men.

“…It is impossible to isolate the contribution of either of these factors but it is likely that the technical problem of fixation and resolution of the tissue plays the major role. This has been noted previously in cilia from 10 "normal" subjects (22) when only 31.3% of the expected total number of dynein arms could be observed. This was considerably less than the number we recorded and could be because four of the subjects in the other study had chronic sinusitis and one had allergic rhinitis.…”

“…Fox et al (22) observed that 4% of cilia did not have the 9 + 2 microtubular arrangement and Rossman et al (15) found 1.7% of cilia with an abnormal number of doublet microtubules and 0.6% with abnormal number of central pair microtubules. We found 4.1 and 1.3%, respectively.…”

“…Tannic acid has been used to resolve individual microtubular protofilaments (20), whereas MgSO4 has been shown to improve the visibility of dynein arms (21,22). By altering the concentration of MgSO4 and tannic acid it has been possible to find a combination of these two compounds which dramatically enhances the fixation, staining, and resolution of axonemal ultrastructure, in particular the dynein arms and radial spokes (Fig.…”

Mutations ofRPGR in X-linked retinitis pigmentosa (RP3)

“…Abnormalities in cilia have been reported in X-linked and autosomal types of RP [Arden and Fox, 1979;Fox et al, 1980;Hunter et al, 1988]. The localization of RPGR to the rod and cone photoreceptor connecting cilium [Hong et al, 2000] raises the possibility that the observed ciliary abnormalities in XLRP are associated with RPGR mutations.…”

Mucociliary Clearance in Bronchiectasis

“…Various investigators have reported abnormalities of nasal cilia in patients with bronchiectasis under electron microscope. [8][9][10] Neutrophil elastase is a serine proteinase that has been implicated in the pathogenesis of bronchiectasis, emphysema, and adult respiratory distress syndrome. It leads to mucous gland hyperplasia, increased airway secretion, damage of the ciliated epithelium, and acceleration of airway inflammation.…”