Yeast models of human mitochondrial diseases.

Abstract:

:The yeast Saccharomyces cerevisiae is an excellent model for gaining insights into the molecular basis of human mitochondrial disorders, particularly those resulting from impaired mitochondrial metabolism. Yeast is a very well characterized system and most of our current knowledge about mitochondrial biogenesis in humans derives from yeast genetics and biochemistry. Systematic yeast genome-wide approaches have allowed for the identification of human disease genes. In addition, the functional characterization of a large number of yeast gene products resident in mitochondria has been instrumental for the later identification and characterization of their human orthologs. Here I will review the molecular and biochemical characterization of several mitochondrial diseases that have been ascribed to mutations in genes that were first found in yeast to be necessary for the assembly of the mitochondrial respiratory chain. The usefulness of yeast as a model system for human mitochondrial disorders is evaluated.

journal_name

IUBMB Life

journal_title

IUBMB life

authors

Barrientos A

doi

10.1002/tbmb.718540876

keywords:

subject

Has Abstract

pub_date

2003-02-01 00:00:00

pages

83-95

issue

2

eissn

1521-6543

issn

1521-6551

journal_volume

55

pub_type

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