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Related Experiment Video

Updated: Jun 10, 2026

Preparation of Mitochondria from Ovarian Cancer Tissues and Control Ovarian Tissues for Quantitative Proteomics Analysis

Preparation of Mitochondria from Ovarian Cancer Tissues and Control Ovarian Tissues for Quantitative Proteomics Analysis

Published on: November 18, 2019

The mitochondrial proteome and human disease.

Sarah E Calvo¹, Vamsi K Mootha

¹Broad Institute of MIT and Harvard, Cambridge, Massachusetts 02142, USA.

Annual Review of Genomics and Human Genetics

|August 10, 2010

Summary

The human mitochondrial proteome, comprising over 1,000 proteins, is crucial for understanding mitochondrial diseases, many of which stem from nuclear DNA defects, not just mitochondrial DNA mutations.

Area of Science:

Biochemistry
Genetics
Molecular Biology

Background:

The human mitochondrial genome (mtDNA) has been studied for decades, primarily for maternally inherited diseases.
However, most mitochondrial disorders arise from nuclear genome defects, as mtDNA encodes only 13 proteins.

Purpose of the Study:

To review recent advancements in characterizing the mammalian mitochondrial proteome.
To highlight insights into the proteome's complexity, heterogeneity, evolutionary origins, and biochemical functions.
To discuss the proteome's application in discovering genetic causes of respiratory chain disorders and defining mitochondrial disease.

Main Methods:

Genomics
Mass spectrometry
Computational analysis

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Last Updated: Jun 10, 2026

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Main Results:

Systematic identification of over 1,000 proteins in the mammalian mitochondrial proteome is now feasible.
Characterization reveals complexity, tissue-specific variations, evolutionary insights, and diverse biochemical roles.
The mitochondrial proteome is instrumental in identifying genetic bases for respiratory chain disorders.

Conclusions:

Understanding the mitochondrial proteome is essential for diagnosing and defining mitochondrial diseases.
Future research directions include systems-level analysis of mitochondria using proteomic data.
The mitochondrial proteome offers a foundation for a comprehensive understanding of mitochondrial function and disease.