Table 2.

B cells isolated from GSL storing mice show disease specific increases in GSLs. Splenocytes were dispersed by gentle disruption between two frosted glass microscope slides, and B cells were isolated using magnetic beads. After GSL extraction and purification, GSLs were digested with ceramide glycanase before derivatisation and quantitation by HPLC. Data are from 4–6 experiments and are expressed as pmol/mg protein ± S.E.

Mouse	LacCer	GA2	Gb3	GM3	GM2	Gb4	GA1	GalNAcGA1	GM1a	GM1b	GD1a
C57B16	280±80	40±l0	70±15	–	40±10	40±10	90±20	30±3	40±9	200±50	240±65
α-Gal−/−	180±90	60±35	2,200±560	–	30±10	30±10	90±30	40±10	30±10	190±70	300±90
β-Gal−/−	160±80	260±20	120±40	–	70±8	60±30	1,700±200	50±10	110±30	740±100	660±90
β-Hexb−/−	360±l00	870±60	50±2	–	90±30	340±50	240±60	30±3	130±40	180±30	260±40
NPC1 ++	260±40	40±3	30±1	6±1	35±3	30±3	70±6	40±3	20±1	150±20	180±20
NPC1 +/−	520±200	85±l0	30±3	6±1	50±6	30±5	80±10	35±5	35±5	160±20	190±30
NPC1 −/−	1,800±450	110±20	140±30	110±3	150±30	160±40	270±60	80±10	70±10	270±40	310±40

LacCer Lactosylceramide, GA2 GalNAcβ4Galβ4GlcCer, Gb3 Galα4Galβ4GlcCer, GM3 Neu5Acα3Galβ4GlcCer, GM2 GalNAcβ4(Neu5Acα3) Galβ4GlcCer, Gb4 GalNAcβ4Galα4Galβ4GlcCer, GA1 Galβ4GalNAcβ4Galβ4GlcCer, GMla Galβ3GalNAcβ4(Neu5Acα3)Galβ4GlcCer, GMlb Neu5Acα3Galβ3GalNAcβ4Galβ4GlcCer, GDla Neu5AcαGalβ3GalNAcβ4(Neu8Acα3)Galβ4GlcCer