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Case Study Of Mitochondria

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Mitochondria are important organelle which is responsible for regulating cellular energy homeostasis and cell death. Hence, the damaged mitochondria could be removed by a mechanism called Mitophagy, which is has particular autophagic elimination mitochondrial (Youle and Narendra, 2011).as the mitochondria are targeted for autophagic degradation (Springer and Macleod, 2016). Furthermore, Mitophagy plays a significant role in cellular homeostasis by eliminating dysfunctional mitochondria and decreasing mitochondrial mass as a response stress. Recent work has linked defect in mitophagy to human diseases such as metabolic disorders diseases (Youle and Narendra, 2011). Furthermore, Metabolic myopathies are inherited or obtained defects in…show more content…
Thus, this study led to different thought as TCAI levels did not increase in McArdle disease for different reasons such as, the direct link between TCAI changes in muscle tissue related to the changes in plasma which is due to the enzymatic deficiency in McArdle disease to skeletal muscle. (Delaney et al., 2017) The key feature of the study designed is based on the comparison of Mito and McArdle patients, which allowed us to determine that restricted TCA cycle expansion is a result of blocked glycogenolysis, which is not a secondary effect of impaired oxidative phosphorylation. Furthermore, pantothenate increases with exercise in mito and control, but not in McArdle patients. And based on pervious work on exaggerated AMP degradation with exercise in McArdle patients showed that metabolites related to the urea cycle are also raised (Delaney et al., 2017). As AMP produce ammonia and the rise in ammonia been part of diagnostic feature in McArdle disease. Basically, the increased ammonia in those patients, related to limited ATP for pyrimidine nucleotide phosphorylation and to increased pyrimidine degradation (Delaney et al., 2017). Furthermore, their measurements in this study presents an important exercise-induced decline in orotate in controls and Mito decrease in orotate in controls as well as Mito patients while in McArdle patients remained uncahnged. The recent study provides additional support for raised creatine and reduce uridine
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