Chemical chaperones increase the cellular activity of N370S beta -glucosidase: A therapeutic strategy for Gaucher disease -- Data Supplement - Supporting Text -- Proceedings of the National Academy of Sciences

Supporting information for Sawkar et al. (2002) Proc. Natl. Acad. Sci. USA, 10.1073/pnas.192582899

Supporting Text
Structural characterization
N
-nonyl-2,5-anhydro-imino-D-glucitol (2). ¹H (300 MHz, CD3OD) d 4.00 (m, 1H), 3.96 (t, 1H, J = 4.2 Hz), 3.71 (ABX, 1H, J_AB= 10.6 Hz, J_AX = 6.1 Hz, J_BX = 4.0 Hz, n_a = 1113.1 Hz, n_b = 1091.8 Hz), 3.64 (ABX, 1H, as above), 3.62 (m, 2H), 3.04 (ABX, 1H, n_X = 9112.4 Hz), 2.71 (m, 3H), 1.51 (quint, 2H, J = 7.1 Hz), 1.31 (m, 12H) 0.91 (t, 3H, J = 6.5 Hz); ¹³C NMR (75 MHz, CD3OD) d 78.2, 77.7, 71.8, 67.2, 62.3, 61.6, 55.5, 33.0, 30.7, 30.4, 28.5, 28.4, 23.7, 14.5; High-resolution mass for C15H31NO4 [ M+H^+]: calculated 290.2326, found 290.2325. 4-Nonylmorpholine (3).
¹H (300 MHz, CDCl₃) d 3.66 (t, 4H, J = 4.7 Hz), 2.38 (t, 4H, J = 4.7 Hz), 2.26 (m, 2H), 1.44 (quint, 2H, J = 7.5 Hz), 1.23 (m, 12H), 0.84 (m, 3H); ¹³C NMR (75 MHz, CDCl₃) d 66.8, 59.1, 53.7, 31.9, 29.6, 29.5, 29.3, 27.5, 26.6, 22.7, 14.1; High-resolution mass for C13H27NO [ M+H^+]: calculated 214.2165, found 214.2163. 1-(Hydroxyethyl)-4-nonylpiperazine (4).
¹H (400 MHz, CDCl₃) d 3.94 (s, 1H), 3.57 (t, 2H, J = 5.6 Hz), 2.50 (t, 4H, J = 5.8 Hz), 2.45 (m, 6H), 2.27 (t, 2H, J = 8.1 Hz), 1.42 (quint, 2H, J = 8.1), 1.21 (m, 12H), 0.82 (t, 3H, J = 7.0); ¹³C NMR (100 MHz, CDCl₃) d 59.3, 58.6, 57.6, 53.0, 52.7, 31.7, 29.4, 29.4, 29.1, 27.4, 26.6, 22.5, 13.9; High-resolution mass for C15H32NO [ M+H^+]: calculated 257.2587, found 257.2581. (2S,3S)-4-hydroxy-1-nonyl-
L-proline (5). ¹H (400 MHz, CD3OD) d 4.58 (m, 1H), 3.85 (m, 1H), 3.80 (s, 1H), 3.37 (td, 1H, J = 11.6, 6.9 Hz), 3.22 (m, 2H), 2.01 (m, 2H), 1.72 (quint, 2H, J = 7.5 Hz), 1.33 (m, 12H), 0.90 (m, 3H); ¹³C NMR (100 MHz, CD3OD) d 171.0, 59.3, 58.6, 57.6, 53.0, 52.7, 31.7, 29.4, 29.4, 29.1, 27.4, 26.6, 22.5, 13.9; High-resolution mass for C14H27NO3 [ M+H^+]: calculated 258.2064, found 258.2063. Diethyl nonylamine.
¹H (300 MHz, CDCl₃) d 2.25 (q, 4H, J = 7.6 Hz), 2.40 (m, 2H), 1.44 (quint, 2H, J = 7.6 Hz), 1.27 (m, 12H), 1.03 (t, 6H, J = 7.1 Hz), 0.89 (t, 3H, J = 6.8 Hz); ¹³C NMR (75 MHz, CDCl₃) d 53.0, 46.9, 32.0, 29.8, 29.7, 29.4, 27.9, 27.0, 22.8, 14.2, 11.8; High-resolution mass for C13H29N [ M+H^+]: calculated 200.2373, found 200.2368. N
-octyl deoxynojirimycin. ¹H (400 MHz, CD3OD) d 3.85 (m, 2H), 3.47 (ddd, 1H, J = 10.6, 9.0, 4.9 Hz), 3.35 (t, 1H, J = 9.2 Hz), 3.13 (t, 1H, J = 9.2 Hz), 2.99 (dd, 1H, J = 11.1, 4.8 Hz), 2.79 (m, 1H), 2.58 (m, 1H), 2.18 (t, 1H, J = 10.9 Hz), 2.16 (dt, 1H, J = 9.6, 2.6 Hz), 1.49 (quint, 2H, J = 7.8 Hz), 1.31 (m, 10H), 0.90 (t, 3H, J = 6.9 Hz); ¹³C NMR (100 MHz, CD3OD) d 80.5, 72.0, 70.7, 67.3, 59.3, 57.6, 53.8, 32.9, 30.6, 30.4, 28.6,

25.1, 23.7, 14.4

; High-resolution mass for C14H29NO4 [ M+H^+]: calculated 276.2169, found 276.2168. Acetal derivative of N-nonyl-2,5-anhydro-imino-D-glucitol (6).
¹H (400 MHz, CD3OD) d 4.13 (d, 1H, J = 4.4 Hz), 4.00 (ABX, 1H, J_AB= 12.4 Hz, J_AX = 3.4 Hz, J_BX = 2.9 Hz, n_a = 1595.9 Hz, n_b = 1491.3 Hz), 3.96 (m, 1H), 3.74 (ABX, 1H, as above), 3.63 (ABX, 1H, J_AB= 10.9 Hz, J_AX = 7.6 Hz, J_BX = 6.5 Hz, n_a = 1446.0 Hz, n_b = 1426.9 Hz), 3.58 (ABX, 1H, as above), 2.84 (q, 1H, J = 3.5 Hz), 2.73 (ddd, 1H, 9.6, 4.6, 2.3 Hz), 2.65 (m, 2H), 1.49 (quint, 2H, J = 7.7 Hz), 1.42 (s, 3H), 1.31 (m, 15H), 0.90 (t, 3H, J = 6.8 Hz); ¹³C NMR (100 MHz, CD3OD) d 98.9, 78.5, 77.7, 74.0, 64.1, 61.6, 61.3, 55.6, 33.0, 30.7, 30.7, 30.4, 29.0, 28.6, 28.5, 23.7, 20.1, 14.4; High-resolution mass for C18H35NO4 [ M+H^+]: calculated 330.2639, found 330.2639.