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Telomeres and Muscular Dystrophy

Telomeres and Muscular Dystrophy

anthony-loera anthony-loera
4 minute read

Telomeres and Muscular Dystrophy?

Telomeres Study: Duchenne muscular dystrophy, or DMD, is a disease caused by a defective dystrophin gene needed for proper muscle structure and function. DMD is the most common childhood inherited muscular dystrophy, affecting approximately one in every 3500 male boys worldwide. Although early symptoms can be observed in the deterioration of calf muscles and other lower limb muscles, the muscles involved in respiration and muscles of the heart normally claim the lives of those afflicted. Scientists that study DMD use a DMD rodent model where the dystrophin gene is also mutated and nonfunctional. [1][2]

Does Long Telomere Length Affect Muscular Dystrophy In A Positive Manner?

Duchenne muscular dystrophy, or DMD, is a disease caused by a defective dystrophin gene needed for proper muscle structure and function. DMD is the most common childhood inherited muscular dystrophy, affecting approximately one in every 3500 male boys worldwide. Although early symptoms can be observed in the deterioration of calf muscles and other lower limb muscles, it is the muscles involved in respiration and muscles of the heart that normally claim the lives of those afflicted. Scientists that study DMD use a DMD rodent model were the dystrophin gene is also mutated and nonfunctional. However, it has been observed by those in this field of study that the rodents with the defective dystrophin gene do not die of cardiorespiratory failure like their human counterparts that suffer from DMD. In other words, something about DMD mice, even though they have the same defective gene, allows them to be more resistant to cardiac dysfunction than humans. This observation until recently remained a mystery, until they question if Telomere Length Affect Muscular Dystrophy in some manner.

Study Shows Long Telomere Length Affect Muscular Dystrophy Cardio Issues:

A group of scientists from Stanford University has now published their observations and determined what is causing the high cardiovascular mortality in humans suffering from DMD (Nature Cell Biology, July 2013). To understand the researcher’s observations and conclusions it is important to point out one less commonly known fact about whether Telomere Length Affect Muscular Dystrophy in rodents. Although rodents have a shorter lifespan than humans, they have much longer telomeres. The researchers speculated that because the rodent’s heart cells have longer telomeres, they can tolerate cellular stress better than humans. To test this, the scientist created mice that not only had the dystrophin gene mutation but also had shorter telomeres length comparable to humans. The researcher's speculations proved to be correct when they observed that these mice now also suffered from severe cardiovascular malfunctions ranging from problems with ventricular dilation, contraction, and conductance, all combined to accelerated mortality in the mice. [3]

Added Proof That Long Telomere Length Affects Muscular Dystrophy Heart Cells.

As proof of whether Telomere Length Affect Muscular Dystrophy, the researchers took heart cell samples from DMD patients, measured their telomere length, and observed 45% shorter telomeres than their age match controls. As the researchers then stated, these findings support a strong link between the dystrophin gene and telomere length but also very important these results also suggest preventative strategies in delaying the pathologies of this horrible disease may involve better care of telomeres.

TA-65 affects telomere length

In a recent study done at UCLA with the help of RevGenetics, it was proved that TA-65 activated telomerase in the human cells of different people. We regard to any positive news of telomere length as a favorable consideration as we believe that TA-65 affects telomere length through the transient activation of telomerase.

References:

  1. https://www.nature.com/articles/ncb2790
  2. https://www.nature.com/articles/nsmb.2571
  3. https://arvojournals.org/article.aspx?articleid=2579553

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