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CFTR-deficient pigs display peripheral nervous system defects at birth


, : CFTR-deficient pigs display peripheral nervous system defects at birth. Proceedings of the National Academy of Sciences of the United States of America 110(8): 3083-3088

Peripheral nervous system abnormalities, including neuropathy, have been reported in people with cystic fibrosis. These abnormalities have largely been attributed to secondary manifestations of the disease. We tested the hypothesis that disruption of the cystic fibrosis transmembrane conductance regulator (CFTR) gene directly influences nervous system function by studying newborn CFTR(-/-) pigs. We discovered CFTR expression and activity in Schwann cells, and loss of CFTR caused ultrastructural myelin sheath abnormalities similar to those in known neuropathies. Consistent with neuropathic changes, we found increased transcripts for myelin protein zero, a gene that, when mutated, can cause axonal and/or demyelinating neuropathy. In addition, axon density was reduced and conduction velocities of the trigeminal and sciatic nerves were decreased. Moreover, in vivo auditory brainstem evoked potentials revealed delayed conduction of the vestibulocochlear nerve. Our data suggest that loss of CFTR directly alters Schwann cell function and that some nervous system defects in people with cystic fibrosis are likely primary.

US$19.90

PMID: 23382208

DOI: 10.1073/pnas.1222729110


Other references

Jach, M.A., 1994: Elmidae Curtis, 1830 and Elmis Latreille, 1802 Proposed conservation as correct spelling and of feminine gender respectively. Bulletin of Zoological Nomenclature 51(1): 25-27

Pollonara, E.; Giunchi, D.; Chierici, E.; Baldaccini, N.E.ilio, 2003: Evaluation of different censusing methodologies of the stone curlew Burhinus oedicnemus. Avocetta, Suppl. (Numero speciale) 27

Anonymous, 1982: Hepatitis B vaccine for use in selected populations. Fda Drug Bulletin 12(1): 5-5

Kopinga; Tinus; de Jonge WJ; de Vries GC, 1987: Nuclear-spin-lattice relaxation and magnetization of the ferromagnetic S=(1/2 chain system (C6H11NH3)CuBr3: Contribution of magnons and solitons. Physical Review. B, Condensed Matter 36(10): 5398-5410

Kem W.R., 1983: Responses of cerebratulus proboscis muscle to putative neuro transmitters. American Zoologist 23(4): 925

Aguilella-Arzo, M.; García-Celma, J.J.; Cervera, J.; Alcaraz, A.; Aguilella, V.M., 2006: Electrostatic properties and macroscopic electrodiffusion in OmpF porin and mutants. The bacterial porin OmpF found in the outer membrane of E. coli is a wide channel, characterized by its poor selectivity and almost no ion specificity. It has an asymmetric structure, with relatively large entrances and a narrow constriction. By...

Hendley, H., 1897: A Case of Tetanus Treated with Subcutaneous Injections of Corrosive Sublimate: Recovery. British Medical Journal 1(1881): 138-139

Qualmann, C.; Nauck, M.A.; Holst, J.J.; Orskov, C.; Creutzfeldt, W., 1995: Glucagon-like peptide 1 secretion in response to luminal sucrose from the upper and lower gut A study using alpha-glucosidase inhibition. Background: After nutrient ingestion there is an early response of glucagon-like peptide 1 (GLP-1) immunoreactivity, although GLP-1 is mainly produced in endocrine cells from the lower gut (ileum and colon/rectum), suggesting that indirect stimula...

Apfel, H.D.; Levenbraun, J.; Quaegebeur, J.M.; Allan, L.D., 1998: Usefulness of preoperative echocardiography in predicting left ventricular outflow obstruction after primary repair of interrupted aortic arch with ventricular septal defect. Residual left ventricular outflow tract (LVOT) obstruction is a significant problem after repair of interrupted aortic arch (IAA) and ventricular septal defect. Resection of subaortic tissue at the time of primary repair, however, is associated wi...

Matioc, A.A., 2010: Training novice users in bag-valve-mask technique. Prehospital and Disaster Medicine 25(4): 370-371