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. Author manuscript; available in PMC: 2013 Jun 1.
Published in final edited form as: Biochim Biophys Acta. 2011 Sep 16;1817(6):883–897. doi: 10.1016/j.bbabio.2011.09.005

Table 1.

Homologue COX subunits and COX assembly factors in the yeast Saccharomyces cerevisiae and human.

YEAST HUMAN ROLE

GENE PROTEIN GENE PROTEIN
Catalytic core (mtDNA encoded structural subunits)

COX1 Cox1 MTCOXI COX1 Catalytic core subunits
COX2 Cox2 MTCOXII COX2
COX3 Cox3 MTCOXIII COX3

Core protective shield (nDNA encoded structural subunits)

COX4 Cox4 COXVb COX5b Subunits required for COX assembly and function
COX5a Cox5a COXIV-1 COX4-1
COX6 Cox6 COXVa COX5a
COX7 Cox7 COXVIIa COX7a
COX8 Cox8 COXVIIc COX7c
COX9 Cox7a COXVIc COX6c
- - COXVIIb COX7b
-- -- COXVIII COX8

COX12 Cox9 COXVIb COX6b Non-essential subunits
COX13 Cox10 COXVIa COX6a

Expression of catalytic core subunits

MSS116 Mss116 - - Helicase involved in splicing of all COX1 and COB
introns
Component of the RNA degradosome
SUV3 Suv3 - - Helicase involved in COX1 aI5β intron splicing
Stability of intron-containing COX1 and COB transcripts
Component of the RNA degradosome
MRS1 Mrs1 - - Required for COX1 aI5β intron splicing
Required for excision of the COB bI3 intron
MNE1 Mne1 - - Required for COX1 aI5β intron splicing
MSS18 Mss18 - - Required for COX1 aI5β intron splicing
Additional unidentified function
COX24 Cox24 - - Required for splicing of aI2 and aI3 COX1 introns
Required for COX1 mRNA translation
NAM2 Nam2 - - Required for COX1 aI4 intron splicing
CCM1 Ccm1 - - Required for COX1 aI4 intron splicing
PET309 Pet309 Yeast: Translational activator of COX1 mRNA
LRPPRC LRPPRC Human: mitochondrial mRNA stability
MSS51 Mss51 - - Translational activator of COX1 mRNA
Cox1 chaperone required for its stability/
maturation/assembly
YGR021w Ygr021w Yeast: No role on COX biogenesis
TACO1 TACO1 Human: COX1 mRNA translational activator
PET111 Pet111 - - Translational activator of COX2 mRNA
PET54 Pet54 - - Translational activator of COX3 mRNA
Required for COX1 aI5β intron splicing and translation
PET122 Pet122 - - Translational activator of COX3 mRNA
PET494 Pet494 - - Translational activator of COX3 mRNA

Membrane insertion and processing of catalytic core subunits

OXA1 Oxa1 OXA1 OXA1 Membrane insertion of COX subunits, cytochrome b and
ATPase proteolipid
COX20 Cox20 COX20 COX20 Cox2 chaperone. Presentation of Cox2-precursor to the
IMP complex
COX18 Cox18 COX18 COX18 Export of the Cox2 C-terminus tail
MSS2 Mss2 -- -- Export of the Cox2 C-terminus tail
PNT1 Pnt1 -- -- Export of the Cox2 C-terminus tail
IMP1 Imp1 -- -- Responsible for the maturation of precursor Cox2
IMP2 Imp2 IMMP2L IMMP2L Required for the stability and activity of Imp1
SOM1 Som1 -- -- Third component of the yeast IMP complex. It could play
a role in substrate recognition

Copper Metabolism and Insertion into catalytic core subunits

COX17 Cox17 COX17 COX17 Delivery of copper to Sco1 and Cox11
SCO1 Sco1 SCO1 SCO1 Transfer of copper to COX and/or reduction of cysteine
SCO2 SCO2 residues in subunit 2
COX11 Cox11 COX11 COX11 Stable formation of the CuB and Mg centers
COX19 Cox19 COX19 COX19 CX9C proteins. They could play roles in redox control
and copper trafficking in the intermembrane space
COX23 Cox23 COX23 COX23
PET191 Pet191 PET191 PET191
CMC1 Cmc1 CMC1 CMC1
CMC2 Cmc1 CMC2 CMC2

Heme A Biosynthesis

COX10 Cox10 COX10 COX10 Farnesylation of protoheme
COX15 Cox15 COX15 COX15 Conversion of heme o to heme a
YAH1 Yah1 FDX2 FDX2 Collaborates with Cox15 in heme o hydroxylation
ARH1 Arh1 ADR ADR Collaborates with Cox15 in heme o hydroxylation

Assembly / Unknown

COX16 Cox16 COX16 COX16 Unknown function
PET117 Pet117 - -- Unknown function
PET100 Pet100 -- -- Formation of assembly intermediates containing Cox7,
Cox8, and Cox9
SHY1 Shy1 SURF1 SURF1 Catalyzes an assembly step involving Cox1
COX14 Cox14 - - Binds Cox1 and is required for its
stability/maturation/assembly
COA1 Coa1 -- -- Binds Cox1 and is required for its
stability/maturation/assembly
COA2 Coa2 -- -- Required for Cox1 stability/maturation/assembly
COA3/
COX25
Coa3
Cox25
-- -- Binds Cox1 and is required for its
stability/maturation/assembly
CMC3/
COA4
Cmc3
Coa4
-- -- CX9C protein involved in late stages of COX assembly