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Updated: Aug 13, 2026

Transmitochondrial Cybrid Generation Using Cancer Cell Lines
Published on: March 17, 2023
Mitochondrial DNA mutations in renal cell carcinomas revealed no general impact on energy metabolism
D Meierhofer1, J A Mayr, K Fink
1Department of Paediatrics, Paracelsus Private Medical University Salzburg, Muellner Hauptstr. 48, A-5020 Salzburg, Austria.
Abstract:
Previously, renal cell carcinoma tissues were reported to display a marked reduction of components of the respiratory chain. To elucidate a possible relationship between tumourigenesis and alterations of oxidative phosphorylation, we screened for mutations of the mitochondrial DNA (mtDNA) in renal carcinoma tissues and patient-matched normal kidney cortex. Seven of the 15 samples investigated revealed at least one somatic heteroplasmic mutation as determined by denaturating HPLC analysis (DHPLC). No homoplasmic somatic mutations were observed. Actually, half of the mutations presented a level of heteroplasmy below 25%, which could be easily overlooked by automated sequence analysis. The somatic mutations included four known D-loop mutations, four so far unreported mutations in ribosomal genes, one synonymous change in the ND4 gene and four nonsynonymous base changes in the ND2, COI, ND5 and ND4L genes. One renal cell carcinoma tissue showed a somatic A3243G mutation, which is a known frequent cause of MELAS syndrome (mitochondrial encephalomyopathy, lactic acidosis, stroke-like episode) and specific compensatory alterations of enzyme activities of the respiratory chain in the tumour tissue. No difference between histopathology and clinical progression compared to the other tumour tissues was observed. In conclusion, the low abundance as well as the frequently observed low level of heteroplasmy of somatic mtDNA mutations indicates that the decreased aerobic energy capacity in tumour tissue seems to be mediated by a general nuclear regulated mechanism.
Insights
Somatic mitochondrial DNA mutations are present in renal cell carcinoma but are often at low levels. These findings suggest nuclear-regulated mechanisms contribute to reduced energy production in tumors.
Area of Science:
- Oncology
- Mitochondrial Biology
- Genetics
Background:
- Renal cell carcinoma (RCC) tissues show reduced respiratory chain components.
- Oxidative phosphorylation alterations may link to tumor development.
Purpose of the Study:
- To investigate somatic mutations in mitochondrial DNA (mtDNA) in RCC.
- To explore the relationship between mtDNA mutations and tumorigenesis.
Main Methods:
- Screening of mtDNA for mutations in RCC tissues and matched normal kidney cortex.
- Utilizing denaturing HPLC analysis (DHPLC) for mutation detection.
- Analyzing mutation heteroplasmy levels, including those below 25%.
Main Results:
- Seven of 15 RCC samples had at least one somatic heteroplasmic mtDNA mutation.
- No homoplasmic somatic mutations were found; many mutations had low heteroplasmy (<25%).
- Identified known D-loop mutations, novel ribosomal gene mutations, and nonsynonymous changes in respiratory complex genes (ND2, COI, ND4, ND5, ND4L).
- One RCC case exhibited the A3243G mutation, associated with MELAS syndrome.
Conclusions:
- Low abundance and heteroplasmy of somatic mtDNA mutations suggest they are not the primary driver of decreased aerobic energy capacity in RCC.
- Nuclear-regulated mechanisms likely mediate the reduced energy metabolism observed in tumor tissues.
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