Abstract
Context: Mitochondria play a vital role in producing the energy needed for different cellular activities.
The role of mitochondria in different diseases and the aging process is gradually being clarified.
Different studies have suggested that mitochondrial dysfunction due to mutations in genes that
maintain the integrity of mitochondrial DNA (mtDNA), mitophagy, and apoptosis can lead to many
neurological and muscular phenotypes as well as diseases in other organ systems including liver,
gastrointestinal tract, heart, and kidneys. We examined the current knowledge of mitochondrial
dysfunction and its role in renal pathophysiology. Additionally, we examined how chronic kidney
diseases can lead to mitochondrial dysfunction through oxidative stress accumulation, which can
subsequently lead to other pathological complications.
Evidence Acquisitions: Directory of Open Access Journals (DOAJ), Google Scholar, PubMed (NLM),
LISTA (EBSCO), and Web of Science have been searched.
Results: The renal pathological manifestation of mitochondrial dysfunction includes tubular defects,
focal segmental glomerular sclerosis (FSGS), glomerular dysfunction, interstitial nephritis, and
cystic kidney disease or renal tumors. These conditions can be caused by mutations in the nuclear
genes that are involved in mtDNA replication and transcription or due to mtDNA mutations in the
genes involved in the respiratory chain.
Conclusions: Clearly, mtDNA plays an important role in renal pathology, and mitochondria may
serve as a potential therapeutic target to treat different renal pathologies.