GM1 Type II and control plasma NMR profiles. (a) Typical 700 MHz 1H NMR spectral profiles from GM1 Type II gangliosidosis and corresponding control plasma (blue and red spectra respectively). Assignments: [1] very-low-density-lipoprotein (vLDL)/low-density-lipoprotein (LDL) triacylglycerol (TAG)-terminal-CH3 functions; [2] BCAAs (valine-, leucine- and isoleucine-CH3s); [3] vLDL/LDL-bulk-chain-(-CH2-)n; [4] Lactate-CH3; [5] Alanine-CH3; [6] lipoprotein TAG-CH2CH2CO- [7] unassigned multiplet; [8] Acetate-CH3 [22] acute-phase glycoprotein-carbohydrate side-chain N-aceytylsugar-CH3/lipoprotein TAG-CH2-CH=CH-; [53] glutamine-β-CH2; [9] lipoprotein TAG-CH2-CO2-; [54] acetone-CH3; [55] acetoacetate-CH3; [15] glutamine-γ-CH2; [10] citrate-CH2A/B; [12] lipoprotein TAG-CH=CH-CH2-CH=CH-/citrate-CH2A/B; [28] albumin lysine residue-ε-CH2; [21] free lysine-ε-CH2/creatinine-N-CH3/creatine-N-CH3; [26] high-density-lipoprotein phospholipid choline head-group-N+(CH)3; [56] β-Glucose-C2H; [24] unassigned multiplet; [11,14,17,18,25] glucose-C2–6H ring protons; [57] unassigned multiplet; [20] tyrosine-/histidine-/phenylalanine-α-CH’s; [27] lactate-CH; [58] threonine-α-CH; [59] β-glucose-C1H; [60] α-glucose-C1H; [61] lipoprotein TAG-CH=CH-; [62] urea-CO-NH2; [29,30] tyrosine aromatic-CH protons; [33,41] histidine-imidazole ring-CHs; [32,36] phenylalanine aromatic-CH’s; [41] Histidine-CH [63] formate-CH. (b) Average blood plasma 1H NMR profiles of unfasted GM1 Type II gangliosidosis patients (n = 10) (blue) and fasted control participants (n = 27) (red). Left: elevated vLDL/LDL TAG- bulk-chain-(-CH2-)n resonance intensities in healthy controls (δ = 1.10–1.30 ppm)/ Right: elevated unsaturated TAG-CH=CH- resonance intensities in healthy controls (δ = 5.10–5.40 ppm). The Supplementary Materials section provides details regarding ethical approval and informed consent of participants, 1H NMR sample preparation and acquisition parameters.