Cilia with defective radial spokes. A cause of human respiratory disease

J. M. Sturgess, J. Chao, John Wong, N. Aspin, J. A. Turner

Research output: Contribution to journalArticle

Abstract

The authors studied the fine structure of respiratory-tract cilia in three siblings with chronic respiratory disease, comparing them with those from a patient with Kartagener's syndrome who had dynein-deficient cilia and with control patients who had chronic bronchitis or chronic sinusitis. Electron microscopy of the siblings revealed a new abnormality in the ciliary axoneme - namely, lack of the radial spokes. Their cilia showed an eccentric central pair of tubules but otherwise had a normal central sheath, outer-doublet microtubules, nexin links and dynein arms. The cilia were immotile. Mucociliary clearance was completely lacking in the three siblings and in the patient with Kartagener's syndrome, but was normal in their parents and unaffected siblings. Sperm from the male sibling showed identical structural abnormalities and were immotile. The authors consider the radial-spoke defect to be the congenital anomaly responsible for dysfunction of the mucociliary clearance mechanism in these three patients and of the immotile sperm in one of them. This defect is apparently another cause of the 'immotile-cilia syndrome.'

Original languageEnglish (US)
Pages (from-to)53-56
Number of pages4
JournalNew England Journal of Medicine
Volume300
Issue number2
StatePublished - 1979
Externally publishedYes

Fingerprint

Cilia
Siblings
Kartagener Syndrome
Mucociliary Clearance
Dyneins
Spermatozoa
Ciliary Motility Disorders
Axoneme
Chronic Bronchitis
Sinusitis
Respiratory System
Electron Microscopy
Chronic Disease
Parents
Immotile Cilia Syndrome Due To Defective Radial Spokes

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Sturgess, J. M., Chao, J., Wong, J., Aspin, N., & Turner, J. A. (1979). Cilia with defective radial spokes. A cause of human respiratory disease. New England Journal of Medicine, 300(2), 53-56.

Cilia with defective radial spokes. A cause of human respiratory disease. / Sturgess, J. M.; Chao, J.; Wong, John; Aspin, N.; Turner, J. A.

In: New England Journal of Medicine, Vol. 300, No. 2, 1979, p. 53-56.

Research output: Contribution to journalArticle

Sturgess, JM, Chao, J, Wong, J, Aspin, N & Turner, JA 1979, 'Cilia with defective radial spokes. A cause of human respiratory disease', New England Journal of Medicine, vol. 300, no. 2, pp. 53-56.
Sturgess, J. M. ; Chao, J. ; Wong, John ; Aspin, N. ; Turner, J. A. / Cilia with defective radial spokes. A cause of human respiratory disease. In: New England Journal of Medicine. 1979 ; Vol. 300, No. 2. pp. 53-56.
@article{da455cbb0150446490c052269d0936d4,
title = "Cilia with defective radial spokes. A cause of human respiratory disease",
abstract = "The authors studied the fine structure of respiratory-tract cilia in three siblings with chronic respiratory disease, comparing them with those from a patient with Kartagener's syndrome who had dynein-deficient cilia and with control patients who had chronic bronchitis or chronic sinusitis. Electron microscopy of the siblings revealed a new abnormality in the ciliary axoneme - namely, lack of the radial spokes. Their cilia showed an eccentric central pair of tubules but otherwise had a normal central sheath, outer-doublet microtubules, nexin links and dynein arms. The cilia were immotile. Mucociliary clearance was completely lacking in the three siblings and in the patient with Kartagener's syndrome, but was normal in their parents and unaffected siblings. Sperm from the male sibling showed identical structural abnormalities and were immotile. The authors consider the radial-spoke defect to be the congenital anomaly responsible for dysfunction of the mucociliary clearance mechanism in these three patients and of the immotile sperm in one of them. This defect is apparently another cause of the 'immotile-cilia syndrome.'",
author = "Sturgess, {J. M.} and J. Chao and John Wong and N. Aspin and Turner, {J. A.}",
year = "1979",
language = "English (US)",
volume = "300",
pages = "53--56",
journal = "New England Journal of Medicine",
issn = "0028-4793",
publisher = "Massachussetts Medical Society",
number = "2",

}

TY - JOUR

T1 - Cilia with defective radial spokes. A cause of human respiratory disease

AU - Sturgess, J. M.

AU - Chao, J.

AU - Wong, John

AU - Aspin, N.

AU - Turner, J. A.

PY - 1979

Y1 - 1979

N2 - The authors studied the fine structure of respiratory-tract cilia in three siblings with chronic respiratory disease, comparing them with those from a patient with Kartagener's syndrome who had dynein-deficient cilia and with control patients who had chronic bronchitis or chronic sinusitis. Electron microscopy of the siblings revealed a new abnormality in the ciliary axoneme - namely, lack of the radial spokes. Their cilia showed an eccentric central pair of tubules but otherwise had a normal central sheath, outer-doublet microtubules, nexin links and dynein arms. The cilia were immotile. Mucociliary clearance was completely lacking in the three siblings and in the patient with Kartagener's syndrome, but was normal in their parents and unaffected siblings. Sperm from the male sibling showed identical structural abnormalities and were immotile. The authors consider the radial-spoke defect to be the congenital anomaly responsible for dysfunction of the mucociliary clearance mechanism in these three patients and of the immotile sperm in one of them. This defect is apparently another cause of the 'immotile-cilia syndrome.'

AB - The authors studied the fine structure of respiratory-tract cilia in three siblings with chronic respiratory disease, comparing them with those from a patient with Kartagener's syndrome who had dynein-deficient cilia and with control patients who had chronic bronchitis or chronic sinusitis. Electron microscopy of the siblings revealed a new abnormality in the ciliary axoneme - namely, lack of the radial spokes. Their cilia showed an eccentric central pair of tubules but otherwise had a normal central sheath, outer-doublet microtubules, nexin links and dynein arms. The cilia were immotile. Mucociliary clearance was completely lacking in the three siblings and in the patient with Kartagener's syndrome, but was normal in their parents and unaffected siblings. Sperm from the male sibling showed identical structural abnormalities and were immotile. The authors consider the radial-spoke defect to be the congenital anomaly responsible for dysfunction of the mucociliary clearance mechanism in these three patients and of the immotile sperm in one of them. This defect is apparently another cause of the 'immotile-cilia syndrome.'

UR - http://www.scopus.com/inward/record.url?scp=0018341965&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0018341965&partnerID=8YFLogxK

M3 - Article

C2 - 152870

AN - SCOPUS:0018341965

VL - 300

SP - 53

EP - 56

JO - New England Journal of Medicine

JF - New England Journal of Medicine

SN - 0028-4793

IS - 2

ER -