Category:LBS/Biosynthesis/CeramideDeg
ceramide | ceramidase fatty acid |
sphingosine | S1P phosphatase |
sphingosine 1-phosphate |
S1P lyase phosphoethanolamine |
hexadecenal | |
hexadecanal | |
palmitate |
ER, Golgi and Lysosomes |
Enzyme function in the Human Genome | |||
---|---|---|---|
Protein name | Gene name (Uniprot) | 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 | Localized to the Golgi complex and highly expressed in placenta. | |
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 | |
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