. 2011 Jun 11;69(1):149–163. doi: 10.1007/s00018-011-0743-1

Table 2.

Summary of systematic analysis of molecular consequences of mutations in ATP7A at 37 and 30°C

ATP7A	Expression^a				Interaction^b		Localization^c						Cu transporting capacity^d
	37°C	30°C	MG132	COMMD1	ATOX1	COMMD1	37°C		30°C		COMMD1		37°C	30°C	COMMD1
	37°C	30°C	MG132	COMMD1	ATOX1	COMMD1	Basal	Cu	Basal	Cu	Basal	Cu	37°C	30°C	COMMD1
WT	+	+++	+++	++	+	+	TGN	PM	TGN	PM	TGN	PM	100%	100%	100%
L873R	+++	ND	ND	+++	+	+	CP, PM	PM	ND	ND	CP, PM	PM	~0%	ND	NS
C1000R	–	+++	++	++	+	+	TGN	TGN	CP	CP	CP	CP	~0%	~80%	~30%
N1304S	++	ND	ND	+++	+	+	CP, PM	CP, PM	ND	ND	CP, PM	CP, PM	~0%	ND	~35%
A1362D	−	+++	++	+++	+	+	TGN	TGN, CP	CP, PM	CP, PM	CP, PM	CP, PM	~0%	~90%	NS

TGN Trans-Golgi network, CP cell periphery, PM plasma membrane, ND not determined, NS non-significant

^aATP7A mutant protein expressions are compared to WT expression (+) after incubation at 37, 30°C, after proteasomal inhibition by MG132 or by expression of COMMD1, respectively

^bInteractions of ATP7A mutants with ATOX1 and COMMD1 are compared to their interaction with ATP7A WT protein (+)

^cSubcellular localization of ATP7A WT and mutant proteins in excess copper, at 37, 30°C, or by expression of COMMD1, respectively

^dCopper exporting capacities of mutant ATP7A proteins are relative to WT function (set at 100%) and are based on the relative induction of the copper reporter at 37, 30°C or after co-expression of COMMD1 (all in presence of 40 μM CuCl2)