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Biochemistry · Biomolecules and enzymes: dynein defect in primary ciliary dyskinesia
Sample itemA young man has chronic sinusitis, recurrent bronchiectasis, dextrocardia and infertility because his sperm are immotile. Electron microscopy of his cilia is most likely to show absence of which structure?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
D. Dynein arms on the outer microtubule doublets
Kartagener syndrome is a form of primary ciliary dyskinesia in which the dynein arms of the axoneme are defective. Ciliary and sperm tail movement need ATP-driven sliding of the microtubule doublets by dynein. Immotile cilia fail to clear mucus and fail to establish normal left-right asymmetry in the embryo.
Option by option
A. Kinesin light chains in the basal body
Kinesin carries cargo along microtubules and is not the axonemal motor of the cilium.
B. Keratin filaments in the ciliary shaft
Keratin filaments are epithelial intermediate filaments and do not power ciliary beating.
C. Actin filaments in the ciliary core
Actin filaments are not the motor of the ciliary axoneme.
D. Dynein arms on the outer microtubule doublets (correct)
Correct. Dynein arms drive microtubule sliding, and their absence causes immotile cilia and sperm with situs inversus.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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