ASMD / Xenpozyme (olipudase alfa) QSP v2.0 mechanistic SBML scaffold. Five-layer architecture (PK; substrate; biomarkers; tissue burden; clinical observables). Includes ADA neutralisation module and paediatric height-Z catch-up rate rule. Built by IQANOVA AI-QSP Master Pipeline v2.0 (Stage 2). AI-generated mechanistic model anchored to published ASCEND, ASCEND-OLE and ASCEND-Peds summary statistics. Not the original Sanofi industry model. Not validated for clinical or regulatory use without further qualification.

 Olipudase_lysosome 1 ADA_neutralisation_potency ADA_titre REA_baseline 1 REA_baseline Olipudase_effective 1 Olipudase_effective 100 SM_spleen_burden baseline_spleen_MN 1 100 SM_liver_burden baseline_liver_MN 1 100 LysoSM_plasma LysoSM0 1 100 Ceramide_plasma Ceramide0 1 DLCO_gain_max_pct 0 baseline_lung_burden SM_lung_burden 0 baseline_lung_burden SM_lung_burden baseline_lung_burden lung_resolution_max_frac 100 platelet_gain_max_frac 0 baseline_spleen_MN SM_spleen_burden 0 baseline_spleen_MN SM_spleen_burden baseline_spleen_MN spleen_resolution_max_frac height_z height_z0 k_tissue_to_plasma Olipudase_tissue k_plasma_to_tissue Olipudase_plasma k_plasma_clearance Olipudase_plasma ADA_neutralisation_potency ADA_titre Olipudase_plasma k_plasma_to_tissue Olipudase_plasma k_tissue_to_plasma Olipudase_tissue k_tissue_to_lysosome Olipudase_tissue k_tissue_to_lysosome Olipudase_tissue k_lysosome_degradation Olipudase_lysosome k_ADA_generation Olipudase_plasma k_ADA_clearance ADA_titre k_SM_input ASM_activity_scale ASM_active_fraction kcat_ASM SM_lysosome Km_SM SM_lysosome k_LysoSM_generation SM_lysosome k_LysoSM_clearance LysoSM_plasma k_ceramide_generation_from_SM ASM_active_fraction kcat_ASM SM_lysosome Km_SM SM_lysosome ceramide_burden_coupling SM_spleen_burden SM_lung_burden SM_liver_burden spleen_burden0 lung_burden0 liver_burden0 Ceramide0 0.05 k_ceramide_clearance Ceramide_plasma k_spleen_resolution ASM_active_fraction SM_spleen_burden 1 spleen_resolution_max_frac baseline_spleen_MN k_liver_resolution ASM_active_fraction SM_liver_burden 1 liver_resolution_max_frac baseline_liver_MN k_lung_resolution ASM_active_fraction SM_lung_burden 1 lung_resolution_max_frac baseline_lung_burden k_height_catchup height_z_asymptote height_z time 0 Olipudase_plasma dose_amount time 14 Olipudase_plasma dose_amount time 28 Olipudase_plasma dose_amount time 42 Olipudase_plasma dose_amount time 56 Olipudase_plasma dose_amount time 70 Olipudase_plasma dose_amount time 84 Olipudase_plasma dose_amount time 98 Olipudase_plasma dose_amount time 112 Olipudase_plasma dose_amount time 126 Olipudase_plasma dose_amount time 140 Olipudase_plasma dose_amount time 154 Olipudase_plasma dose_amount time 168 Olipudase_plasma dose_amount time 182 Olipudase_plasma dose_amount time 196 Olipudase_plasma dose_amount time 210 Olipudase_plasma dose_amount time 224 Olipudase_plasma dose_amount time 238 Olipudase_plasma dose_amount time 252 Olipudase_plasma dose_amount time 266 Olipudase_plasma dose_amount time 280 Olipudase_plasma dose_amount time 294 Olipudase_plasma dose_amount time 308 Olipudase_plasma dose_amount time 322 Olipudase_plasma dose_amount time 336 Olipudase_plasma dose_amount time 350 Olipudase_plasma dose_amount time 364 Olipudase_plasma dose_amount