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Longen S, et al.  (2009) Systematic analysis of the twin cx(9)c protein family. J Mol Biol 393(2):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.

Status: Published Type: Journal Article PubMed ID: 19703468

Topics addressed in this paper

Number of different genes curated to this paper: 85

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Topics Genes linked to topics (#1 - 10 )
AIR1 AIR2 ARG81 ARO80 ASG1 ASH1 BCD1 CAT8 CHA4 CMC1
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Topics Genes linked to topics (#11 - 20 )
CMC2 CMC4 COA4 COX17 COX19 COX23 CRS5 CUP1-1 CUP1-2 DAL81
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Topics Genes linked to topics (#21 - 30 )
ECM22 EDS1 EMI1 ERV1 FAP1 FKS1 GAL4 GIS2 GSC2 HAL9
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Topics Genes linked to topics (#31 - 40 )
HEL1 HEM2 ILV2 ITT1 LEU3 LYS14 MAC1 MAL13 MAL33 MAP1
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Topics Genes linked to topics (#41 - 50 )
MDM35 MIA40 MIC14 MIC17 MRP10 MSL5 NTO1 OAF1 PET191 PIP2
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Topics Genes linked to topics (#51 - 60 )
PPR1 PUF2 PUT3 RAM1 RDR1 RDS1 RDS3 RGA1 RGT1 SDH2
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Topics Genes linked to topics (#61 - 70 )
SEF1 SIP4 SOM1 STB4 STB5 SUT1 SYG1 TBS1 TEA1 TYW1
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Topics Genes linked to topics (#71 - 80 )
UBR1 UGA3 UGA4 UME6 UPC2 URC2 WAR1 YCR015C YER184C YFL052W
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Topics Genes linked to topics (#81 - 85 )
YIL046W-A YJL206C YLL054C YLR278C YPR196W
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