Table 4.
List of lipid species involved in dysregulated lipid metabolism in NCL disease.
| NCL type | Lipid class | Lipid species | Level | Refs |
|---|---|---|---|---|
| CLN1 | Phospholipids | LBPA (36:2; 34:1); PC (32:1, 34:1) | High | 37–39,42 |
| PC (32:1, 34:1); PI (36:3, 38:5, 38:3); PC (32:1, 34:1) | ||||
| PE (38:5, 36:4, 38:5, 36:2, 34:1, 34:2) | ||||
| PS (34:4, 38:3, 40:3, 34:1, 36:1, 36:2, 38:2) | ||||
| LBPA (38:1); PC (38:4, 38:6) | Low | |||
| PE (40: 6, 38:4, 40:4); PI (38:4, 40:6); PS (40:6) | ||||
| Sphingolipids | SM (16:0, 16:1, 18:1) | High | 38 | |
| SM (20:0) | Low | |||
| CLN2 | Phospholipids | GPE (18:1) | High | 43 |
| GPE (16:0, 18:0) | Low | |||
| CLN3 | Phospholipids | LPE (20:3); PC (32:1, 34:1, 36:3) | High | 37,40 |
| PS (38:3); PI (38:3); PE (38:3, 40:3) | ||||
| PA (36:1, 36:2); PC (38:4); LPI (18:0, 20:4); LPC (20:4) | Low | |||
| PG (34:2, 34:1); PE (40:4); PI (36:1, 36:2, 36:3, 36:4, 36:5) | ||||
| Sphingolipids | Cer (16:0, 24:0, 24:1); SM (24:1) | High | 39,40 | |
| GM3 (d18:1/24:1, d18:1/16:0, d18:1/24:0) | ||||
| GM3 (d18:1/24:1, d18:1/16:0, d18:1/24:0) | ||||
| GD1 (d18:1/25:0, d18:1/16:0, d18:1/22:0); SM (14:0, 15:0) | Low | |||
| HexCer (22:0, 20:0, 18:0, 23:0); LacCer (16:0, 24:0) | ||||
| Sterols | CE (18:2, 18:3) | Low | 40 | |
| CLN9 | Sphingolipids | Cer (16:0, 24:0, 24:1) | Low | 37,39 |
| dhCer (16:0, 24:0, 24:1) |
Cer: ceramide; CholE: cholesterol Ester; dhCer: dihydroceramides; GD1: monosialoganglioside D1; GM3: monosialoganglioside GM3; GPE: glycerophosphoethanolamine; HexCer: hexosylceramide; LacCer: lactosylceramide; LBPA: lysobisphosphatidic acid; LPC: lysophosphatidylcholine; LPE: lysophosphatidylethanolamine; LPI: lysophosphatidylinositol; PA: phosphatidic acid; PC: phosphatidylcholine; PE: phosphatidylethanolamine; PG: phosphatidylglycerol; PI: phosphatidylinositol; PS: phosphatidylserine; SM: sphingomyelin.