°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 |