Document Detail

Role of adenylate kinase type 7 expression on cilia motility: possible link in primary ciliary dyskinesia.
MedLine Citation:
PMID:  20537283     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Adenylate kinase 7 (AK7) mediates the reaction 2ADP <--> ATP + AMP, providing energy for the beating of cilia. A study recently showed that AK7 expression may be correlated with the primary ciliary dyskinesia (PCD) phenotype in mice. In this study, we characterized AK7 expression in vitro in an air-liquid interface (ALI) model and in middle nasal turbinate biopsy specimens from a cohort of patients with PCD to elucidate whether AK7 expression is correlated with ciliary malfunction.
METHODS: AK7 expression was measured by real-time reverse-transcription polymerase chain reaction and Western blotting. In vitro differentiated nasal human epithelial cell siRNA experiments were performed to investigate the effect of AK7 expression on ciliary beat frequency (CBF). Ciliary motility and ultrastructure were evaluated in a cohort of 29 patients with PCD (PCD, n = 17; Kartagener's syndrome, n = 12) and 26 healthy control donors.
RESULTS: AK7 expression was mainly located on the apical surface of differentiated nasal ALI cells, and targeted suppression of the AK7 gene decreased CBF by 41%. AK7 expression was diminished significantly in patients with PCD (0.54 +/- 0.1-fold; p < 0.05) compared with healthy controls (1.1 +/- 0.08-fold). Furthermore, AK7 expression was correlated with CBF in patients with PCD (r = 0.5; p = 0.009).
CONCLUSION: AK7 expression was correlated with CBF in vitro and in nasal biopsy specimens from patients with PCD, which may have contributed to the ciliary malfunction observed in our patients with PCD.
Javier Milara; Miguel Armengot; Manuel Mata; Esteban J Morcillo; Julio Cortijo
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  American journal of rhinology & allergy     Volume:  24     ISSN:  1945-8932     ISO Abbreviation:  Am J Rhinol Allergy     Publication Date:    2010 May-Jun
Date Detail:
Created Date:  2010-06-11     Completed Date:  2010-11-15     Revised Date:  2011-11-10    
Medline Journal Info:
Nlm Unique ID:  101490775     Medline TA:  Am J Rhinol Allergy     Country:  United States    
Other Details:
Languages:  eng     Pagination:  181-5     Citation Subset:  IM    
Research Unit, University General Hospital Consortium, Avenida tres cruces s/n, Valencia, Spain.
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MeSH Terms
Adenylate Kinase / genetics,  metabolism*
Blotting, Western
Cell Differentiation
Cell Movement
Cells, Cultured
Cilia / metabolism*,  ultrastructure*
Energy Metabolism
Kartagener Syndrome / genetics,  metabolism*,  pathology,  physiopathology
Mucociliary Clearance / genetics
RNA, Small Interfering / genetics
Reverse Transcriptase Polymerase Chain Reaction
Turbinates / pathology,  surgery
Reg. No./Substance:
0/RNA, Small Interfering; EC Kinase

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