. Author manuscript; available in PMC: 2013 Sep 23.

Published in final edited form as: J Inherit Metab Dis. 2010 May 11;33(3):261–270. doi: 10.1007/s10545-010-9109-3

Table 3.

Total GSL and cholesterol accumulation in storage disease B cells. B cells were purified from splenocytes. After lipid extraction, GSLs were purified by HPLC and cholesterol was quantitated using a microassay. Data are from 4–6 experiments and are expressed as nmol/mg

Mouse	Total GSL	%	Total cholesterol	%
C57Bl6	1.5±0.3	100	60±8	100
α-Gal^−/−	2.7±0.5	180	110±50	180
β-Gal^−/−	3.8±0.5	250	160±100	260
β-Hexb^−/−	3.7±0.5	240	270±90	450
NPC1⁺⁺	1.3±0.2	100	100±40	160
NPC1^+/−	1.3±0.2	100	60±20	100
NPC1^−/−	5.4± 1.3	400	410±100	680