+ Resolve Article
+ Follow Us
Follow on FacebookFollow on Facebook
Follow on TwitterFollow on Twitter

+ Translate
+ Subscribe to Site Feed
GeoScience Most Shared ContentMost Shared Content

Technical knockout, a Drosophila model of mitochondrial deafness

, : Technical knockout, a Drosophila model of mitochondrial deafness. Genetics 159(1): 241-254

Mutations in mtDNA-encoded components of the mitochondrial translational apparatus are associated with diverse pathological states in humans, notably sensorineural deafness. To develop animal models of such disorders, we have manipulated the nuclear gene for mitochondrial ribosomal protein S12 in Drosophila (technical knockout, tko). The prototypic mutant tko25t exhibits developmental delay, bang sensitivity, impaired male courtship, and defective response to sound. On the basis of a transgenic reversion test, these phenotypes are attributable to a single substitution (L85H) at a conserved residue of the tko protein. The mutant is hypersensitive to doxycyclin, an antibiotic that selectively inhibits mitochondrial protein synthesis, and mutant larvae have greatly diminished activities of mitochondrial redox enzymes and decreased levels of mitochondrial small-subunit rRNA. A second mutation in the tko gene, Q116K, which is predicted to impair the accuracy of mitochondrial translation, results in the completely different phenotype of recessive female sterility, based on three independent transgenic insertions. We infer that the tko25t mutant provides a model of mitochondrial hearing impairment resulting from a quantitative deficiency of mitochondrial translational capacity. Reprinted by permission of the publisher.


PMID: 11560901

Other references

Hunter, D.M.; Moorhouse, D.E., 1976: Comparative bionomics of adult Austrosimulium pestilens Mackerras & Mackerras and A. bancrofti (Taylor) (Diptera, Simuliidae). In studies in Queensland, large numbers of adults of Austrosimulium pestilens Mack. & Mack. were found to appear about a week after floods in permanent and temporary waterways. There was only short period when stream flow remained suitable for ovi...

Ingkaninan, K.; Ijzerman, A.P.; Taesotikult, T.; Verpoorte, R., 2000: Isolation of opioid-active compounds from Tabernaemontana pachysiphon leaves. A procedure for prefractionation of crude plant extracts by centrifugal partition chromatography (CPC) has been developed to enable rapid identification of known-positive compounds or false-positive compounds and to increase the chance of identify...

Sajo, M.D.s-G.M.nezes, N.-De, 1986: Anatomical studies of the roots of species of Vernonia Screb. (Compositae) from Serra do Cipo (Minas Gerais, Brazil). Hoehnea 13(13): 51-58

Chung S.J., 1990: Formulas predicting the percentile of heart weights by body weight in subjects from birth to 19 years of age. The values reported by Scholz and his coworkers [Mayo Clin Proc, 63 (1988) 126-136,637] show that distributions of normal heart weights in the period of body growth from birth to 19 years of age are age and sex dependent and actually more dependen...

Tamura, T.; Hayakawa, M.; Hatano, K., 2001: A new genus of the order Actinomycetales, Virgosporangium gen. nov., with descriptions of Virgosporangium ochraceum sp. nov. and Virgosporangium aurantiacum sp. nov. Four motile spored strains were isolated from soil samples collected in Japan. The cultures formed long, narrow sporangia on shorts sporangiophores directly on the substrate mycelium. The sporangia develop singly or in clusters above the surface o...

Barkley, M.D.; Lewis, P.A.; Sullivan, G.E., 1981: Ion effects on the lac repressor--operator equilibrium. The effects of ions on the interaction of lac repressor protein and operator DNA have been studied by the membrane filter technique. The equilibrium association constant was determined as a function of monovalent and divalent cation concentrations...

Freeman, R., 2014: MHRA recommendations on the use of intravenous acetylcysteine in paracetamol overdose. Archives of Disease in Childhood. Education and Practice Edition 99(1): 37-40

Thrall, R.S.; Guernsey, L.; Manzo, L.; Kindel, S.; Cloutier, M.M., 2002: Increase scar formation in an ovalbumin-induced mouse model of allergic airway disease may be influenced by epithelial cell TGFbeta synthesis. Journal of Allergy & Clinical Immunology 109(1 Supplement): S73, January

Neary, J.P.; Malbon, L.; McKenzie, D.C., 2002: Relationship between serum, saliva and urinary cortisol and its implication during recovery from training. This study examined the relationship among resting cortisol levels measured in serum (SER), saliva (SAL). overnight urine (ON) and 24 h urinary free cortisol (24 h) samples to determine which would be most appropriate for monitoring recovery from...

Anonymous, 2012: Study shows benefit of new maintenance therapy for multiple myeloma. Multiple myeloma is a form of cancer where the plasma cells in the bone marrow grow out of control, causing damage to bones as well as predisposing patients to anemia, infection and kidney failure. Unfortunately, multiple myeloma continues to prog...