Category:LBS/Biosynthesis/CeramideDeg: Difference between revisions
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| Acid ceramidase | | Acid ceramidase | ||
| ASAH1 | | [http://www.uniprot.org/uniprot/Q13510 ASAH1] | ||
| | | 8p22 | ||
| Localized in lysosome. Its deficiency causes lysosomal accumulation of ceramides (Farber disease).<ref>Ferlinz K, Kopal G, Bernardo K et al. (2001) "Human acid ceramidase: processing, glycosylation, and lysosomal targeting" J Biol Chem 276(38):35352-35360.</ref> | | Localized in lysosome. Its deficiency causes lysosomal accumulation of ceramides (Farber disease).<ref>Ferlinz K, Kopal G, Bernardo K et al. (2001) "Human acid ceramidase: processing, glycosylation, and lysosomal targeting" J Biol Chem 276(38):35352-35360.</ref> | ||
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| Neutral ceramidase | | Neutral ceramidase | ||
| ASAH2 | | [http://www.uniprot.org/uniprot/Q9NR71 ASAH2] | ||
| | | 10q11.23 | ||
| Localized to the plasma membrane and expressed in the small intestine and colon. <ref>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</ref> | | Localized to the plasma membrane and expressed in the small intestine and colon. <ref>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</ref> | ||
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|rowspan="3"| Alkaline ceramidase | |rowspan="3"| Alkaline ceramidase | ||
| ACER1 | | [http://www.uniprot.org/uniprot/Q8TDN7 ACER1](ASAH3) | ||
| | | 19p13.3 | ||
| Localized to the endoplasmic reticulum (ER) and highly expressed in skin. | | Localized to the endoplasmic reticulum (ER) and highly expressed in skin. | ||
|- | |- | ||
| ACER2 | | [http://www.uniprot.org/uniprot/Q5QJU3 ACER2](ASAH3L) | ||
| | | 9p22.1 | ||
| Localized to the Golgi complex and highly expressed in placenta. | | Localized to the Golgi complex and highly expressed in placenta. | ||
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| ACER3 | | [http://www.uniprot.org/uniprot/Q9NUN7 ACER3](APHC) | ||
| | | 11q13.5 | ||
| Localized to the ER and the Golgi complex, and ubiquitously expressed. | | Localized to the ER and the Golgi complex, and ubiquitously expressed. | ||
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| rowspan="2"| S1P phosphatase | | rowspan="2"| S1P phosphatase | ||
| SGPP1 | | [http://www.uniprot.org/uniprot/Q9BX95 SGPP1] | ||
| | | 14q23.2 | ||
| Specifically dephosphorylates sphingosine 1-phosphate (S1P), dihydro-S1P, and phyto-S1P. | | Specifically dephosphorylates sphingosine 1-phosphate (S1P), dihydro-S1P, and phyto-S1P. | ||
|- | |- | ||
| SGPP2 | | [http://www.uniprot.org/uniprot/Q8IWX5 SGPP2] | ||
| | | 2q36.1 | ||
| Localized to the ER and dephosphorylates S1P and dihydro-S1P.<ref>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.</ref> | | Localized to the ER and dephosphorylates S1P and dihydro-S1P.<ref>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.</ref> | ||
|- | |- | ||
| S1P lyase | | S1P lyase | ||
| SGPL1 | | [http://www.uniprot.org/uniprot/O95470 SGPL1] | ||
| | | 10q22.1 | ||
| | | | ||
|- | |- | ||
|colspan="4"|<references/> | |colspan="4"|<references/> | ||
|} | |} | ||
Latest revision as of 06:32, 18 December 2017
| 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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