. 2010 May 20;34(1):39–48. doi: 10.1007/s10545-010-9087-5

Table 2.

Effects of δ-ALA, betaine, glycerol and taurine on amounts of tetramers/oligomers of 27 CBS mutants

topology of the mutants	mutants	δ-ALA		betaine		glycerol		taurine
topology of the mutants	mutants	0 mM	0.5 mM	0 mM	100 mM	0 mM	750 mM	0 mM	50 mM
buried mutants	p.G305R	213.4^a	277.8^a	137.2^a	187.2^a	276.6	264.8	199.8	237.8
	p.G307S	250.2^a	311.9^a	230.2	226.4	102.5^a	143.1^a	258.7	253.3
	p.P422L	86.0^a	100.5^a	28.2	27.2	23.5^a	47.3^a	19.0	20.0
	p.I278T	N.D.	N.D.	N.D.	N.D.	N.D.	N.D.	N.D.	N.D.
	p.E144K	N.D.	N.D.	N.D.	N.D.	N.D.	N.D.	N.D.	N.D.
	p.G148R	N.D.	N.D.	N.D.	N.D.	1.9^a	20.8^a	N.D.	N.D.
	p.R266K	28.7^a	65.8^a	15.0^a	19.3^a	11.8^a	28.6^a	11.1	8.4
	p.T262R	N.D.	N.D.	N.D.	N.D.	N.D.	N.D.	N.D.	N.D.
	p.N228K	N.D.	N.D.	N.D.	N.D.	N.D.	N.D.	N.D.	N.D.
	p.L539S	N.D.	N.D.	N.D.	N.D.	N.D.	N.D.	N.D.	N.D.
	p.W409_G453del	N.D.	N.D.	N.D.	N.D.	N.D.	N.D.	N.D.	N.D.
	p.C165Y	152.3^a	270.2^a	112.4^a	155.7^a	125.3^a	172.3^a	100.5	109.9
	p.S466L	133.0^a	199.0^a	47.1	55.0	53.4^a	74.9^a	88.7	81.8
solvent accessible mutants	p.H65R	N.D.	N.D.	N.D.	N.D.	4.6^a	18.8^a	N.D.	N.D.
	p.R369C	9.7^a	47.7^a	17.7^a	19.7^a	10.9^a	19.4^a	19.1	17.8
	p.T191M	N.D.	N.D.	N.D.	N.D.	N.D.	N.D.	N.D.	N.D.
	p.A114V	127.3^a	207.5^a	69.6	59.2	113.4	130.2	127.0	115.9
	p.P78R	45.9^a	72.6^a	7.2^a	13.1^a	9.7^a	34.0^a	4.2	4.2
	p.R125Q	7.3^a	64.2^a	6.4^a	13.6^a	15.1^a	22.9^a	6.5	7.8
	p.K102N	24.2^a	45.4^a	6.5^a	9.3^a	8.4^a	22.7^a	12.6	15.6
	p.E176K	5.8	2.2	13.7	14.2	12.6^a	29.2^a	7.3	6.8
	p.V180A	41.9^a	76.6^a	11.6^a	16.2^a	12.5^a	30.9^a	20.6	19.9
	p.I435T	72.3	50.9	76.4^a	109.8^a	125.8^a	186.8^a	109.1	96.7
	p.P49L	224.3^a	256.0^a	160.0^a	198.2^a	88.2^a	109.3^a	156.1	163.1
	p.E302K	175.8^a	233.8^a	247.5	213.2	234.1	218.0	187.4	182.8
	p.R439Q	148.2	153.5	219.1	247.3	329.2	233.1	189.4	204.8
	p.D444N	180.1	208.4	261.0	297.8	377.6	350.8	291.0	300.5
	wild-type CBS	100.0^a	110.2^a	100.0^a	109.3^a	100.0^a	120.6^a	100.0^a	105.4^a

ND not detected, signal of CBS antigen on Western blot below the signal of negative control

^aSignals of responsive mutants (>30%) and wild-type expressed as percent of signal of wild-type CBS as a mean of two independent experiments