KEY RESOURCES TABLE

REAGENT or RESOURCE	SOURCE	IDENTIFIER
Antibodies
Mouse monoclonal anti-Por11	Abcam	Cat# ab110326; RRID:AB_10865182
Mouse monoclonal anti-Cox2	Abcam	Cat# ab110271; RRID:AB_10858117
Rabbit polyclonal anti-Coa6	Gohil Lab	N/A
Rabbit polyclonal anti- SCO1	Bourens et al., 2014	N/A
Rabbit polyclonal anti- SCO2	Fitzgerald	Cat# 70R-20118
Rabbit Polyclonal anti- COA6	Proteintech	Cat# 24209–1-AP
Mouse monoclonal anti- COX2	Mitoscience	Cat# MS405; RRID:AB_1618186
Rabbit monoclonal anti- GAPDH	Cell signaling	Cat# 14C10–2118
hFAB Rhodamine anti-Actin	Bio-Rad	Cat# 12004163
Bacterial and Virus Strains
E. coli strain DH5α	NEB	Cat# C2987I
E. coli BL21(DE3)	NEB	Cat# C2527I
Chemicals, Peptides, and Recombinant Proteins
Ammonium-15N chloride	Sigma-Aldrich	Cat# 299251
BME Vitamins 100x solution	Sigma-Aldrich	Cat# B6891
D-(+)-Raffinose pentahydrate	Sigma-Aldrich	Cat# R0250
D-Glucose-13C6	Sigma-Aldrich	Cat# 389374
DL-Dithiothreitol	Sigma-Aldrich	Cat# D0632
Ergosterol	Sigma-Aldrich	Cat# 45480
Isopropyl β-D-thiogalactoside	Sigma-Aldrich	Cat# I6758
Trans-4,5-Dihydroxy-1,2-dithiane (Oxidized DTT)	Sigma-Aldrich	Cat# D3511
Deposited Data
Human COA6 structure	This Paper	PDB: 6NL3
Experimental Models: Cell Lines
COA6 patient cell line	Baertling et al., 2015	N/A
SCO1–1 (R149X/P174L) patient cell line	Valnot et al., 2000	N/A
SCO1–2 (V93X/M294V) patient cell line	Leary et al., 2013	N/A
SCO2–9 patient cell line	Leary et al., 2013	N/A
Experimental Models: Organisms/Strains
S. cerevisiae: Strain background: BY4741 WT - MATa, his3Δ1, leu2Δ0, met15Δ0, ura3Δ0	Miriam L. Greenberg	N/A
S. cerevisiae: Strain background: BY4741 coa6Δ - MATa, his3Δ1, leu2Δ0, met15Δ0, ura3Δ0 coa6Δ::kanMX4	Open Biosystems	N/A
S. cerevisiae: Strain background: BY4741 sco1Δ - MATa, his3Δ1, leu2Δ0, met15Δ0, ura3Δ0 sco1Δ::kanMX4	Open Biosystems	N/A
S. cerevisiae: Strain background: BY4741 sco2Δ - MATa, his3Δ1, leu2Δ0, met15Δ0, ura3Δ0 sco2Δ::kanMX4	Open Biosystems	N/A
S. cerevisiae: Strain background: BY4741 cox11Δ - MATa, his3Δ1, leu2Δ0, met15Δ0, ura3Δ0 cox11Δ::kanMX4	Open Biosystems	N/A
S. cerevisiae: Strain background: BY4741 cox17Δ - MATa, his3Δ1, leu2Δ0, met15Δ0, ura3Δ0 cox17Δ::kanMX4	Open Biosystems	N/A
S. cerevisiae: Strain background: STY10 sco2Δcoa6Δ - MATa, his3Δ1, leu2Δ0, ura3Δ0, lys2Δ0, sco2Δ::KanMX4, coa6Δ::NatMX4	Ghosh et al., 2016	N/A
Recombinant DNA
pGEX4t-1- COA6 isoform3	This paper	N/A
pET28a- Coa6	This paper	N/A
pETG-30A- SCO1	Banci et al., 2006	N/A
pETG-30A- SCO2	Banci et al., 2007	N/A
pET9a-COX2-chimeric protein	Morgada et al., 2015	N/A
pRS416 -hyCOA6	Ghosh et al., 2016	N/A
pRS416 -hyCOA6w26C	Soma et al., 2018	N/A
pRS416 -hyCOA6w33R	Soma et al., 2018	N/A
pRS416 -hyCOA6E54X	Soma et al., 2018	N/A
pRS416 -hyCOA6(Δ65–79)	This paper	N/A
pRS416 -hyCOA6(Δ73–79)	This paper	N/A
Software and Algorithms
The PyMOL Molecular Graphics System, v2.0	Schrödinger LLC	https://pymol.org/2/
HADDOCK2.2	van Zundert and Bonvin, 2014	http://haddock.science.uu.nl/services/HADDOCK2.2/
Topspin, version 3.2	Bruker, Germany	N/A
Sparky, version 3.112	UCSF	https://www.cgl.ucsf.edu/home/sparky/distrib-3.112/download.html
CYANA software package	Güntert and Buchner, 2015	https://www.las.jp/english/products/cyana.html
TALOS+	Shen et al., 2009	https://spin.niddk.nih.gov/bax/nmrserver/talos/
Xplor NIH version 2.50	Schwieters et al., 2003	https://nmr.cit.nih.gov/xplor-nih/
Protein Structure Validation Software suite 1.5	Bhattacharya et al., 2007	https://psvs-1_5-dev.nesg.org/