Paola is a 3-year-old girl with a disease that reduces the ability of her mitochondria to generate ATP. Explain the specific effects of this disease on the ability of Paola's muscles to function properly. What other tissues and organs are likely to be especially affected by her disease, and why? (Connects to ​Chapter 3​)

Video transcript

Welcome back, everybody. Let's take a look at our next problem. Mitochondrial storage diseases can affect the number and function of a person's mitochondria. Which of the following statements best describes the consequence of having such a disease. A ATP production will remain stable and unaffected b, fatigue, weakness, weakness, metabolic strokes, seizures, cardiomyopathy and arrhythmias are some of the symptoms common in mitochondrial storage diseases. C when the number or function of mitochondria, the cell is disrupted, more energy is produced or d the disease can be passed on by males. Well, hopefully, we can rule out twisty right away. Since we recall that mitochondria are inherited only from the mother, they're passed on in the egg cell and mitochondrial DNA is passed only from mother to child. So, mitochondrial diseases cannot be passed on by males to their Children. So choice d rule that out right away. So now let's think about what mitochondria do. Mitochondria. Of course, their most important and main function is to provide energy for the cells and they provide that energy in the form of A TP. So if you have a mitochondrial towards disease, your ability to produce a TP and store it when needed will be affected. Obviously, this will affect all the cells of the body, particularly the ones that have very high energy needs, such as the heart, such as muscles such as the brain. So when we look at our answer, choices, choice B makes the most sense. You have all these wide variety of symptoms, fatigue, muscle weakness, strokes, seizures, cardiomyopathy, all these things that get affected because your body can't produce enough energy carrying A TP. When we look at our other answer, choices, choice A says A TP production will remain stable and unaffected, but this is obviously incorrect since mitochondria are the source of that A TP. So if you have a disease affecting your mitochondria, A TP production will not remain stable. And choice C says when the number of function of mitochondria, the cell is disrupted, more energy is produced and that's pretty obviously incorrect. Since again, the problem with the mitochondria will lead to less A TP since their function is to produce it. So once again, the best description of the consequences of having mitochondrial disease is choice B and we have those list of symptoms being common in mitochondrial storage diseases. See you in the next video.