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Researchers generate a mutant mouse model useful in the treatment of neuromuscular diseases
Date:1/29/2013

In three to six months of life, this genetic alteration in mice similar to that occurred in human causes a rapid degeneration in the lower limbs to death for cardiac arrest.

For the first time in the world, researchers at the Center for Biomedical Research of the University of Granada have created mice with a genetic mutation inducing a deficiency in the coenzyme Q10, a rare mitochondrial disease prevailingly affecting children. These mutant mice which lack the Coq9 gene will be "a valuable tool for the study and treatment of metabolic encephalopathies and neuromuscular diseases", the researchers state.

Coenzyme Q10 (CoQ10) is a molecule produced in body cells, which functions are crucial to cell metabolism. Their best-known function is their generating energy used by cells and their antioxidant activity. In human, defects in the biosynthetic route cause CoQ10 deficiency, resulting in a syndrome with very heterogeneous symptoms.

To better understand the pathological mechanisms of this disease and learn about the biosynthetic pathway of CoQ, the University of Granada researchers conducted a three-year study to generate mice with a mutation in a gene (Coq9) similar to that found in humans. This gene codifies a protein involved in CoQ biosynthesis.

Lower Limb Paralysis

Accordikng to professor Luis Carlos Lpez Garca the principal investigator of this study states, "mice with a Coq9 mutation develop a severe encephalomyopathy inducing neural death, astrogliosis and vacuolation of the brain. At three to six months, these mice undergo a rapid degeneration causing lower limp paralysis and death for cardiac arrest. In molecular terms, CoQ deficiency in mice impairs the mitochondrial mechanisms of bioenergy production in the brain, causing a severe bioenergetic deficiency and a slight increase in oxidative damage".

The CoQ9-deficient mutant mouse model generated at the University of Granada "is the first mo
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Contact: Luis Carlos Lpez Garca
luisca@ugr.es
University of Granada
Source:Eurekalert  

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