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Biomedical subjects

B A Afzelius

Publications and source records attributed to B A Afzelius.

At least 91 records · Page 5Linked to original sources

On the pathogenesis of obstructive lung disease. A study on the immotile-cilia syndrome.

The role of mucociliary transport in man can be evaluated by studying persons with the recently recognised "immotile-cilia syndrome". Such persons have chronic or recurrent infections of the upper and lower airways and have ultrastructural defects of cilia and sperm tails. Men suffering from the syndrome usually are sterile and have immotile spermatozoa. About half of the subjects have complete situs inversus. Fourteen persons with the immotile-cilia syndrome have been studied here in an attempt to evaluate the role of an impaired mucociliary transport for the development of obstructive lung disease. Age range was 25-40 years; there were ten men and four women. Mucociliary transport in the lungs was extremely slow in all subjects. Spirometry demonstrated airway obstruction in nine subjects. Two of these subjects had radiological evidence of pulmonary emphysema. The results indicate that an absent mucociliary transport predisposes to the development of obstructive lung disease. Patients with classic chronic bronchitis have earlier been shown to have a severely impaired mucociliary transport. The present findings in subjects with primary ciliary immotility make it probable that this impaired mucociliary transport is of pathogenetic importance for development of the obstructive lung disease often found in patients with chronic bronchitis.

Adult↗

The immotile-cilia syndrome. A congenital ciliary abnormality as an etiologic factor in chronic airway infections and male sterility.

We investigated six men and a woman suspected of suffering from congenital immotility of cilia. All had chronic airway infections, and the men had immotile spermatozoa. The woman and three men had Kartagener's syndrome. The investigations included measurements of the mucociliary transport in the lower airways and ultrastructural studies of the sperm tails or respiratory cilia (or both). Mucociliary transport was significantly delayed. Sperm tails lacked dynein arms in five patients. Respiratory cilia from the women and two men lacked dynein arms and were irregularly oriented. The results support the hypothesis that a congenital defect in the cilia and sperm tails will cause chronic respiratory-tract infections and male sterility--the immotile-cilia syndrome. In about half these patients there will also be a situs inversus--i.e., Kartagener's syndrome.

Adult↗

Protein of insect sperm mitochondrial crystals. Crystallomitin.

Mitochondrial derivatives of insect sperm usually contain a crystalline protein that shows a 45-nm main period, made up of 20-nm subperiods, determined by the coiling of filament bundles. Filaments are 2 nm thick and have a globular appearance. The crystals contain two main polypeptides, 52,000 and 55,000 daltons. These polypeptides are closely related, contain a high percentage of proline, and are insoluble in sodium dodecyl sulfate due to disulfide cross links. We suggest for this class of protein the name crystallomitin.

Amino Acids↗

A human syndrome caused by immotile cilia.

Four subjects who produced immotile sperm were studied. In three of the subjects, who had frequent bronchitis and sinusitis, there was no mucociliary transport, as measured by tracheobronchial clearance. Electron microscopy indicated that cilia from cells of these patients lack dynein arms.

Bronchitis↗

Calcium and sulfur location in human nail.

Analytical electron microscopy was performed in order to elucidate the importance of calcium and sulfur to the hardness of nails. The free surfaces adsorb a considerable quantity of calcium from the environment. It is suggested that such adsorption occurs due to ion exchange. This concept is supported by the fact that the distribution of sulfur does not vary over the nail cross-section. Analytical electron microscopy offers unique possibilities of elemental distribution analysis in tissue fragments or sections from keratinized tissues.

Calcium↗

Lack of dynein arms in immotile human spermatozoa.

Sermatozoa from two brothers who are not twins were found to be straight and immotile. Examinations of the sperm showed that oxygen consumption and lactic acid production were normal; viability tests showed that the percentage of dead sperm was not increased. The ultrastructural appearance of the sperm tail was normal except for a complete lack of dynein arms and some irregularities in the arrangement of the accessory fibers and the longitudinal columns of the fibrous sheath. The mitochondrial apparatus and the sperm head conform to the conventional model. According to the sliding-filament hypothesis first proposed by Afzelius (1959. J. Biophys. Biochem. Cytol. 5:269.), the arms are responsible for the bending movements of the tail. The simplest explanation for the simultaneous lack of arms and sperm motility appears to be that the two brothers have a genetic disorder involving production, assembly, or attachment of the dynein arms.

Adult↗

Evidence of congenitally nonfunctioning cilia in the tracheobronchial tract in two subjects.

Mucociliary transport in the tracheobronchial tract was studies in 2 subjects by having them inhale a radioactively tagged test aerosol and by taking external measurements of the radioacitivity in the lungs for 2 hours. Both subjects had living, yet immotile seprmatozoa; the sperm tails lacked normal dynein arms, which are essential for the bending movements of sperm tails as well as of other cilia. The subjects were classified as suffering from Kartagener's syndrome (situs inversus, chronic sinusitis, and bronchiectasis) and were shown to have an extremely slow, probably absent transport. The results of the study were in agreement with a recent hypothesis explaining Kartagener's syndrome as due to a genetic lack of dynein arms. An estimate of the importance of mucociliary transport as a protective mechanism of the lung may be possible by examining patients suffering from Kartagener's syndrome.

Adult↗