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PMID:19703468

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Citation

Longen, S, Bien, M, Bihlmaier, K, Kloeppel, C, Kauff, F, Hammermeister, M, Westermann, B, Herrmann, JM and Riemer, J (2009) Systematic analysis of the twin cx(9)c protein family. J. Mol. Biol. 393:356-68

Abstract

The Mia40-Erv1 disulfide relay system is of high importance for mitochondrial biogenesis. Most so far identified substrates of this machinery contain either two cysteine-x(3)-cysteine (twin Cx(3)C) or two cysteine-x(9)-cysteine (twin Cx(9)C) motifs. While the first group is composed of well-characterized components of the mitochondrial import machinery, the molecular function of twin Cx(9)C proteins still remains unclear. To systematically characterize this protein family, we performed a database search to identify the full complement of Cx(9)C proteins in yeast. Thereby, we identified 14 potential family members, which, with one exception, are conserved among plants, fungi, and animals. Among these, three represent novel proteins, which we named Cmc2 to 4 (for Cx(9)C motif-containing protein) and which we demonstrated to be dependent for import on the Mia40-Erv1 disulfide relay. By testing deletion mutants of all 14 proteins for function of the respiratory chain, we found a critical function of most of these proteins for the assembly or stability of respiratory chain complexes. Our data suggest that already early during the evolution of eukaryotic cells, a multitude of twin Cx(9)C proteins developed, which exhibit largely nonredundant roles critical for the biogenesis of enzymes of the respiratory chain in mitochondria.

Links

PubMed Online version:10.1016/j.jmb.2009.08.041

Keywords

Amino Acid Sequence; Computational Biology; Genome, Fungal/genetics; Microscopy, Fluorescence; Mitochondrial Membrane Transport Proteins/chemistry; Mitochondrial Membrane Transport Proteins/genetics; Mitochondrial Membrane Transport Proteins/metabolism; Mitochondrial Proteins/chemistry; Mitochondrial Proteins/genetics; Mitochondrial Proteins/metabolism; Molecular Sequence Data; Open Reading Frames; Oxidoreductases Acting on Sulfur Group Donors/chemistry; Oxidoreductases Acting on Sulfur Group Donors/genetics; Oxidoreductases Acting on Sulfur Group Donors/metabolism; Ribosomal Proteins/chemistry; Ribosomal Proteins/genetics; Ribosomal Proteins/metabolism; Saccharomyces cerevisiae/genetics; Saccharomyces cerevisiae/growth & development; Saccharomyces cerevisiae/metabolism; Saccharomyces cerevisiae Proteins/chemistry; Saccharomyces cerevisiae Proteins/genetics; Saccharomyces cerevisiae Proteins/metabolism; Sequence Homology, Amino Acid

Significance

Annotations

Gene product Qualifier GO Term Evidence Code with/from Aspect Extension Notes Status

YEAST:MDM35

involved_in

GO:0033108: mitochondrial respiratory chain complex assembly

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

YEAST:COXM1

located_in

GO:0005758: mitochondrial intermembrane space

ECO:0000314: direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

YEAST:COX23

involved_in

GO:0033108: mitochondrial respiratory chain complex assembly

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

YEAST:MIX17

involved_in

GO:0009060: aerobic respiration

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

YEAST:MIX14

involved_in

GO:0009060: aerobic respiration

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

YEAST:EMI1

involved_in

GO:0007005: mitochondrion organization

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

YEAST:SOM1

involved_in

GO:0033108: mitochondrial respiratory chain complex assembly

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

YEAST:COA4

involved_in

GO:0033108: mitochondrial respiratory chain complex assembly

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

YEAST:COA4

located_in

GO:0005758: mitochondrial intermembrane space

ECO:0000314: direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

YEAST:COXM2

involved_in

GO:0033108: mitochondrial respiratory chain complex assembly

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

YEAST:COXM2

located_in

GO:0005758: mitochondrial intermembrane space

ECO:0000314: direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

YEAST:CMC4

located_in

GO:0005758: mitochondrial intermembrane space

ECO:0000314: direct assay evidence used in manual assertion

C

Seeded From UniProt

complete


See also

References

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