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Number of Nexin Links Detectable at Standard Electron Microscopy of Normal Human Nasal Cilia and at Nexin Link Deficiency.

Cramnert, Catharina and Stenram, Unne LU (2014) In Ultrastructural Pathology 38(6). p.377-381
Abstract
Abstract Background: Eleven years ago we had described three patients with missing nexin links as a possible cause of primary ciliary dyskinesia (PCD). The assumption was substantiated last year by finding a mutation in these patients. Materials and Methods: We counted the nexin links, inner (IDA) and outer (ODA) dynein arms and microtubuli in each of, if possible, 50 cilia in 41 patients with normal cilia, 4 patients with deficiency of nexin links only and 4 with deficiency of nexin links and IDA. Results: In the control group the median number of nexin links was 4.5 per cilium, range 3.4-5.3. In the second group the mean numbers of nexin links per cilium were 1.1-1.4, in the third group 0.8-1.2, per patient. The median number of IDA was... (More)
Abstract Background: Eleven years ago we had described three patients with missing nexin links as a possible cause of primary ciliary dyskinesia (PCD). The assumption was substantiated last year by finding a mutation in these patients. Materials and Methods: We counted the nexin links, inner (IDA) and outer (ODA) dynein arms and microtubuli in each of, if possible, 50 cilia in 41 patients with normal cilia, 4 patients with deficiency of nexin links only and 4 with deficiency of nexin links and IDA. Results: In the control group the median number of nexin links was 4.5 per cilium, range 3.4-5.3. In the second group the mean numbers of nexin links per cilium were 1.1-1.4, in the third group 0.8-1.2, per patient. The median number of IDA was in the control group 4.2, range 3.3-5.2. In groups 2 and 3 the numbers were 3.0-3.5 and 0.2-1.0, respectively. Numbers of ODA were normal in all groups. Conclusions: It is possible to reliable count the number of nexin links in nasal human cilia and to distinguish cases with missing nexin links from normal controls. (Less)
Please use this url to cite or link to this publication:
author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Ultrastructural Pathology
volume
38
issue
6
pages
377 - 381
publisher
Taylor & Francis
external identifiers
  • pmid:24971518
  • wos:000345348200002
  • scopus:84911460763
ISSN
1521-0758
DOI
10.3109/01913123.2014.930081
language
English
LU publication?
yes
id
25208a57-8d2e-44fb-83cc-cbc016da6310 (old id 4526671)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/24971518?dopt=Abstract
date added to LUP
2014-07-07 21:44:56
date last changed
2017-02-17 11:28:09
@article{25208a57-8d2e-44fb-83cc-cbc016da6310,
  abstract     = {Abstract Background: Eleven years ago we had described three patients with missing nexin links as a possible cause of primary ciliary dyskinesia (PCD). The assumption was substantiated last year by finding a mutation in these patients. Materials and Methods: We counted the nexin links, inner (IDA) and outer (ODA) dynein arms and microtubuli in each of, if possible, 50 cilia in 41 patients with normal cilia, 4 patients with deficiency of nexin links only and 4 with deficiency of nexin links and IDA. Results: In the control group the median number of nexin links was 4.5 per cilium, range 3.4-5.3. In the second group the mean numbers of nexin links per cilium were 1.1-1.4, in the third group 0.8-1.2, per patient. The median number of IDA was in the control group 4.2, range 3.3-5.2. In groups 2 and 3 the numbers were 3.0-3.5 and 0.2-1.0, respectively. Numbers of ODA were normal in all groups. Conclusions: It is possible to reliable count the number of nexin links in nasal human cilia and to distinguish cases with missing nexin links from normal controls.},
  author       = {Cramnert, Catharina and Stenram, Unne},
  issn         = {1521-0758},
  language     = {eng},
  number       = {6},
  pages        = {377--381},
  publisher    = {Taylor & Francis},
  series       = {Ultrastructural Pathology},
  title        = {Number of Nexin Links Detectable at Standard Electron Microscopy of Normal Human Nasal Cilia and at Nexin Link Deficiency.},
  url          = {http://dx.doi.org/10.3109/01913123.2014.930081},
  volume       = {38},
  year         = {2014},
}