ceramide
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ceramidase
 fatty acid
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sphingosine
|
S1P phosphatase

|
sphingosine 1-phosphate
|
S1P lyase
 phosphoethanolamine
|
hexadecenal
|

|
hexadecanal
|

|
palmitate
|
ER, Golgi and Lysosomes
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[show]Enzyme function in the Human Genome
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Protein name
|
Gene name (Uniprot)
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Location
|
|
Acid ceramidase
|
ASAH1
|
8p22
|
Localized in lysosome. Its deficiency causes lysosomal accumulation of ceramides (Farber disease).[1]
|
Neutral ceramidase
|
ASAH2
|
10q11.23
|
Localized to the plasma membrane and expressed in the small intestine and colon. [2]
|
Alkaline ceramidase
|
ACER1(ASAH3)
|
19p13.3
|
Localized to the endoplasmic reticulum (ER) and highly expressed in skin.
|
ACER2(ASAH3L)
|
9p22.1
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Localized to the Golgi complex and highly expressed in placenta.
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ACER3(APHC)
|
11q13.5
|
Localized to the ER and the Golgi complex, and ubiquitously expressed.
|
S1P phosphatase
|
SGPP1
|
14q23.2
|
Specifically dephosphorylates sphingosine 1-phosphate (S1P), dihydro-S1P, and phyto-S1P.
|
SGPP2
|
2q36.1
|
Localized to the ER and dephosphorylates S1P and dihydro-S1P.[3]
|
S1P lyase
|
SGPL1
|
10q22.1
|
|
- ↑ Ferlinz K, Kopal G, Bernardo K et al. (2001) "Human acid ceramidase: processing, glycosylation, and lysosomal targeting" J Biol Chem 276(38):35352-35360.
- ↑ Coant N, Sakamoto W, Mao C, Hannun YA (2015) "Ceramidases, roles in sphingolipid metabolism and in health and disease" Adv Biol Regul 63:122-131
- ↑ Ogawa C, Kihara A, Gokoh M, Igarashi Y (2003) "Identification and characterization of a novel human sphingosine-1-phosphate phosphohydrolase, hSPP2" J Biol Chem 278(2):1268-1272.
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