| °C | degree Celsius |
| ΔΔCt | Delta-Delta-Count-Threshold |
| 3-kdhSP | 3-Keto-dihydro-sphingosine |
| aCDase | acid Ceramidase, encoded by Asah1 |
| ACER | alkaline Ceramidase |
| Acer2 | alkaline Ceramidase 2 (Golgi Ceramidase) |
| Acer3 | alkaline Ceramidase 3 (ER and Golgi Ceramidase) |
| ACTB | Actin-B |
| ALS | Amyotrophic Lateral Sclerosis, spinal motor neuron atrophy at adult age, tauopathy |
| ASAH | N-Acylsphingosine Amidohydrolase (acid or neutral Ceramidase) |
| Asah1 | N-Acylsphingosine Amidohydrolase 1 (lysosomal acid Ceramidase) |
| Asah2 | N-Acylsphingosine Amidohydrolase 2 (plasma membrane/mitoch. neutral Ceramidase) |
| ASMase | acid Sphingomyelinase |
| ATXN2 | Ataxin-2 |
| BSA | Bovine serum albumin |
| C22 chain | Chain with a length comprising 22 carbons |
| CAG | Cytosine-adenine-guanine |
| cDNA | Complementary deoxyribo-nucleic acid |
| Cer | Ceramide |
| Cers1 | Ceramide Synthase 1 (primary in brain, C18 ceramide, ER of neurons, astrocytes and OPC) |
| Cers2 | Ceramide Synthase 2 (very long-chain ceramides, mainly in ER of mature oligodendroglia) |
| CoA | Coenzyme-A |
| CSF | Cerebrospinal fluid |
| d18:0 | Di-hydroxy sphingoid base, 18 carbon chain length, 0 double bonds |
| DEAE | Diethyl-aminoethyl |
| dhSP | Dihydro-sphingosine |
| EDTA | Ethylene-Diamine-Tetra-Acetic acid |
| Elovl1 | Elongation of very long-chain fatty acids protein 1 (oligodendrocyte, C22–26 SFA) |
| Elovl2 | Elongation of very long-chain fatty acids protein 2 (astrocyte, C20–22 PUFA) |
| Elovl3 | Elongation of very long-chain fatty acids protein 3 (eye, cholesterol/odd-chain elongase) |
| Elovl4 | Elongation of very long-chain fatty acids protein 4 (neurons, C24–26 SFA) |
| Elovl5 | Elongation of very long-chain fatty acids protein 5 (astrocyte, C18 PUFA) |
| Elovl6 | Elongation of very long-chain fatty acids protein 6 (ubiquitous, C12–16 PUFA) |
| Elovl7 | Elongation of very long-chain fatty acids protein 7 (oligodendrocyte, C16–20 SFA+PUFA) |
| ER | Endoplasmic reticulum |
| FA | Fatty acid |
| FDA | Federal Drug Administration |
| FTLD | Fronto-temporal lobar degeneration/dementia, cortical motor neuron atrophy, tauopathy |
| GalCer | Galactosyl-ceramide |
| GD1b | Ganglioside 1b with Di-NANA binding |
| GM1a | Ganglioside 1a with Mono-NANA binding |
| GT1b | Ganglioside 1b with Tri-NANA binding |
| h | Hour |
| HCl | Hydrochloric acid |
| HSAN1 | Hereditary sensory and autonomic neuropathy type 1 |
| ILVs | Intralysosomal luminal vesicles |
| K1 | KIN sample 1 |
| KIN | Knockin (of CAG100 mutation into Atxn2 gene, in this case) |
| KO | Knockout (of Atxn2 gene, in this case) |
| LC-MS | Liquid chromatography mass spectrometry |
| MA-nSMase | Mitochondria-associated neutral sphingomyelinase |
| MAPT | Microtubule-associated protein tau |
| µL | Microliter |
| µg | Microgram |
| mg | Milli-gram |
| min | Minute |
| Mito | Mitochondria |
| mRNA | Messenger RNA |
| MSA | Multi-system atrophy |
| MTBE | Methyl-tert-butyl ester |
| mTORC1 | Mechanistic target of rapamycin complex 1, a kinase responsible for cell growth signals |
| Naaa | N-Acylethanolamine acid amidase (acid ceramidase-like protein, mainly in macrophages) |
| NaCl | Sodiumchloride |
| NANA | N-acetyl-neuraminic acid |
| nCDase | Neutral Ceramidase, encoded by Asah2 |
| ng | Nanogram |
| NPA | Niemann–Pick type A, caused by mutations in the aSMase Smpd1, neurovisceral picture |
| NPB | Niemann–Pick type B, caused by mutations in the aSMase Smpd1, visceral picture |
| NPC | Niemann–Pick type C, caused by Npc1/Npc2 mutations, neuronopathic picture |
| NSMase | Neutral Sphingomyelinase |
| nSMase1 | Neutral Sphingomyelinase 1 (encoded by Smpd2) |
| OPC | Ooligodendrocyte precursor cell |
| OPCA | Olivo-ponto-cerebellar atrophy |
| PCR | Polymerase chain reaction |
| PKC | Protein kinase C |
| polyQ | polyGlutamine |
| PSP | Progressive supranuclear palsy (Parkinson plus), dopaminergic neuron atrophy, tauopathy |
| PUFA | Poly-unsaturated fatty acid |
| RHEB | Ras homolog enriched in brain, mTORC1-binding protein |
| RIPA | Radio-immuno precipitation assay |
| RNA | Ribonucleic acid |
| s | Second |
| S1P | Sphingosine-1-phosphate |
| SAPs | sphingolipid activator proteins |
| SCA2 | Spino-cerebellar ataxia type 2, caused by polyQ expansions in ataxin-2 |
| SCA34 | Spino-cerebellar ataxia type 34, caused by inactivity of ELOVL4 |
| SCA38 | Spino-cerebellar ataxia type 38, caused by inactivity of ELOVL5 |
| SDS | Sodium-dodecyl-sulfate |
| s.e.m. | Standard error of the mean |
| SFA | Saturated fatty acid |
| Sgms1 | Sphingomyelin synthase 1 (Golgi location) |
| Sgms2 | Sphingomyelin synthase 2 (plasma membrane location) |
| Smpd1 | Sphingomyelin phosphodiesterase 1 (acid lysosomal SMase) |
| Smpd2 | Sphingomyelin phosphodiesterase 2 (neutral plasma membrane SMase, immune cells) |
| Smpd3 | Sphingomyelin phosphodiesterase 3 (neutral Golgi+ plasma membrane SMase, brain stress) |
| Smpd4 | Sphingomyelin phosphodiesterase 4 (neutral ER/Golgi membrane SMase) |
| Smpd5 | Sphingomyelin phosphodiesterase 5 (ER and mitochondria-associated neutral SMase) |
| SM | Sphingomyelin |
| SMase | Sphingomyelinase |
| SMS | Sphingomyelin synthase |
| SPT | Serine palmitoyltransferase |
| Sptlc1 | Serine palmitoyltransferase long-chain base subunit 1 |
| Sptlc2 | Serine palmitoyltransferase long-chain base subunit 2 (for C18 substrates, in ER) |
| Sptlc3 | Serine palmitoyltransferase long-chain base subunit 3 (for C12-16 substrates) |
| Suppl. | Supplementary |
| Tbp | TATA-binding protein |
| TBS-T | Tris-buffered saline with Tween20 |
| TGN | Trans-Golgi network |
| TLC | Thin layer chromatography |
| TLR2 | Toll-like receptor 2 |
| W1 | WT sample 1 |
| WT | Wild-type |