The impact of metabolic stressors on mitochondrial homeostasis in a renal epithelial cell model of methylmalonic aciduria.

Author: BrutscheMarion, GroßOlaf, GrünertSarah C, HannibalLuciana, HavermansMonique, KölkerStefan, SchumannAnke, SpiekerkoetterUte

Paper Details 
Original Abstract of the Article :
Methylmalonic aciduria (MMA-uria) is caused by deficiency of the mitochondrial enzyme methylmalonyl-CoA mutase (MUT). MUT deficiency hampers energy generation from specific amino acids, odd-chain fatty acids and cholesterol. Chronic kidney disease (CKD) is a well-known long-term complication. We exp...See full text at original site
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引用元:
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10175303/

データ提供:米国国立医学図書館(NLM)

Metabolic Stressors and Mitochondrial Homeostasis in Methylmalonic Aciduria

This research delves into the complex world of [methylmalonic aciduria (MMA-uria)], a rare genetic disorder that affects [mitochondrial function] and can lead to [chronic kidney disease (CKD)]. The authors investigated the impact of [metabolic stressors] on [mitochondrial homeostasis] in [renal epithelial cells] from [MMA-uria patients], traversing the vast and intricate desert of [molecular biology] to understand the underlying mechanisms of this disease.

Metabolic Stressors Aggravate Mitochondrial Dysfunction

The study revealed that [metabolic stressors] such as [high protein intake] and [increased isoleucine/valine levels] further aggravated [mitochondrial dysfunction] in [MMA-uria cells]. These findings provide crucial insights into the [disease pathogenesis] and the potential for [complications] in [MMA-uria patients]. It's like a camel caravan encountering harsh desert conditions, where the already fragile ecosystem of [mitochondria] becomes even more vulnerable.

Understanding the Link Between MMA-uria and CKD

The research sheds light on the potential link between [MMA-uria] and [CKD]. The study found that [metabolic stressors] exacerbated [mitochondrial dysfunction] and [cellular damage] in [MMA-uria cells], providing a clearer understanding of the [disease progression] and the importance of [early intervention]. It's like understanding the intricate relationship between a desert ecosystem and the camels that traverse it, recognizing that certain conditions can lead to a decline in the health of both.

Dr. Camel's Conclusion

This research provides critical insights into the metabolic challenges faced by individuals with methylmalonic aciduria. Understanding the impact of metabolic stressors on mitochondrial homeostasis is crucial for developing effective therapies and preventing the development of chronic kidney disease in these patients.

Date :
  1. Date Completed 2023-05-15
  2. Date Revised 2023-06-01
Further Info :

Pubmed ID

37169781

DOI: Digital Object Identifier

PMC10175303

SNS
PICO Info
in preparation
Languages

English

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