Increased apoptosis of myoblasts in Drosophila model for the Walker-Warburg syndrome.

PloS One
Morio UeyamaShoko Nishihara

Abstract

Walker-Warburg syndrome, a progressive muscular dystrophy, is a severe disease with various kinds of symptoms such as muscle weakness and occasional seizures. The genes of protein O-mannosyltransferases 1 and 2 (POMT1 and POMT2), fukutin, and fukutin-related protein are responsible for this syndrome. In our previous study, we cloned Drosophila orthologs of human POMT1 and POMT2 and identified their activity. However, the mechanism of onset of this syndrome is not well understood. Furthermore, little is known about the behavioral properties of the Drosophila POMT1 and POMT2 mutants, which are called rotated abdomen (rt) and twisted (tw), respectively. First, we performed various kinds of behavioral tests and described in detail the muscle structures by using these mutants. The mutant flies exhibited abnormalities in heavy exercises such as climbing or flight but not in light movements such as locomotion. Defective motor function in mutants appeared immediately after eclosion and was exaggerated with aging. Along with motor function, muscle ultrastructure in the tw mutant was altered, as seen in human patients. We demonstrated that expression of RNA interference (RNAi) for the rt gene and the tw mutant was almost completely letha...Continue Reading

References

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Citations

Jan 27, 2016·Neuromuscular Disorders : NMD·Volker Straub, Marta Bertoli
Apr 2, 2016·Archives of Biochemistry and Biophysics·Kenji MiyamotoYuzuru Ikehara
May 6, 2015·Genes to Cells : Devoted to Molecular & Cellular Mechanisms·Miki Yamamoto-HinoSatoshi Goto
Jul 26, 2011·Biochemical and Biophysical Research Communications·Keiko Akasaka-ManyaTamao Endo
Feb 25, 2017·Insect Biochemistry and Molecular Biology·Tomasz WalskiGuy Smagghe
Apr 11, 2015·Molecules : a Journal of Synthetic Chemistry and Natural Product Chemistry·Emilie PlantiéKrzysztof Jagla
Nov 28, 2019·Glycoconjugate Journal·Shoko Nishihara
Apr 3, 2019·Glycoconjugate Journal·Weidong LiGuy Smagghe
Oct 19, 2019·International Journal of Molecular Sciences·András A KissMátyás Mink

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Methods Mentioned

BETA
gene knockdown
biopsies
dissection
PCR
transgenic
electrophoresis

Software Mentioned

R

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