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What goes wrong with the protein Dynein, that leads to Primary Ciliary Dyskinesia ? |
What goes wrong with the protein Dynein, that leads to Primary Ciliary Dyskinesia ? Dynein is one of the proteins that help the cilia to generate their beating pattern. Each cilium has a central pair of microtubules and an outer set of nine 'doublets' - pairs comprising one complete and one incomplete microtubule, arranged in a circular pattern. Each of these microtubules are attached to the basal body of the cell, and each doublet is also attached to the central pair by radial spokes. it is a defect in the genetic coding of left right dynein in the cilia which lines the respiratory tract. The coding defect is unknown as yet although dynein is a key structural protein in cilia Dynein is a class of protein responsible for the bending of cilia, and Primary Ciliary Dyskinesia is a heterogeneous group of inherited disorders characterised by a structural and generalised abnormality of cilia which renders them immotile or dysmotile. The most common clinical expressions of this condition are chronic upper and lower airway disease and male infertility. ..... Evidence has been found that a genetic defect of the gene(s) for dynein, leading to a congenital lack of dynein arms in cilia and sperm tails, was responsible for both the respiratory and fertility problems. this protein helps cilia(small projections ) present in respiratory tract , genital tract to move and perform its job so when this protein is defected or abscent you will have what we call (immotile cilia syndrome or Primary Ciliary Dyskinesia as you called it .....there are many variants for this problem (like Kartagner syndrome ,young syndrome) |
| Tags |
| Dysphagia Dyspepsia Dyspareunia Dysosmia Dysmenorrhea Dyslexia Dyskinesia Dysgeusia Dysentery Dysautonomia Dwarfism DVT Duodenal Ulcer |
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