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Among all the organelles in an animal cell, only mitochondria have their own independent genomes. Animal mitochondrial DNA is a double-stranded, closed-circular molecule with around 20,000 base pairs. Mitochondrial DNA is unique in that one of its two strands, the heavy, or H, -strand is guanine rich, whereas the complementary strand is cytosine rich and called the light, or L, -strand. Compared to nuclear DNA, mitochondrial DNA has a very low percentage of non-coding regions and is marked by...
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The mitochondrial electron transport chain (ETC) is the main energy generation system in the eukaryotic cells. However, mitochondria also produce cytotoxic reactive oxygen species (ROS) due to the large electron flow during oxidative phosphorylation. While Complex I is one of the primary sources of superoxide radicals, ROS production by Complex II is uncommon and may only be observed in cancer cells with mutated complexes.
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Mitochondria are eukaryotic cellular organelles that are known to produce energy through a process called oxidative phosphorylation. Besides their primary function, mitochondria are involved in various cellular processes, including cell growth, differentiation, signaling, metabolism, and senescence. Age-related changes cause a decline in mitochondrial quality and integrity due to increased mitochondrial mutations and oxidative damage. Thus, aging can severely impact mitochondrial functions,...
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Disruption of Mitochondrial Quality Control in Inherited Metabolic Disorders.

Manuela Bianchin Marcuzzo1, Josyane de Andrade Silveira1, Emílio L Streck2

  • 1Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos, 2600-Anexo, Porto Alegre, RS, 90035-003, Brazil.

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Inherited metabolic disorders (IMDs) involve toxic metabolite buildup. This review examines how mitochondrial quality control defects contribute to IMD pathophysiology in preclinical models.

Keywords:
BiogenesisFissionFusionInherited Metabolic DisordersMitochondrial Quality ControlMitophagy

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Area of Science:

  • Biochemistry
  • Genetics
  • Cell Biology

Background:

  • Inherited metabolic disorders (IMDs) are genetic conditions causing toxic metabolite accumulation.
  • Mitochondrial dysfunction, including impaired citric acid cycle and oxidative stress, is observed in some IMDs.
  • Mitochondrial quality control maintains organelle health through biogenesis, dynamics, and mitophagy.

Purpose of the Study:

  • To review preclinical evidence on mitochondrial quality control disturbances in IMDs.
  • To explore the role of mitochondrial dysfunction in IMD pathophysiology.

Main Methods:

  • Summary of preclinical data from animal models.
  • Analysis of patient-derived cell studies.

Main Results:

  • Preclinical studies indicate mitochondrial quality control defects in selected IMDs.
  • Secondary abnormalities in mitochondrial quality control may contribute to IMD pathogenesis.

Conclusions:

  • Mitochondrial quality control is a potential factor in the pathophysiology of inherited metabolic disorders.
  • Further research in preclinical models can elucidate the role of mitochondrial defects in IMDs.