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Literature summary extracted from

  • Jennemann, R.; Rabionet, M.; Gorgas, K.; Epstein, S.; Dalpke, A.; Rothermel, U.; Bayerle, A.; van der Hoeven, F.; Imgrund, S.; Kirsch, J.; Nickel, W.; Willecke, K.; Riezman, H.; Groene, H.J.; Sandhoff, R.
    Loss of ceramide synthase 3 causes lethal skin barrier disruption (2012), Hum. Mol. Genet., 21, 586-608 .
    View publication on PubMed

Cloned(Commentary)

EC Number Cloned (Comment) Organism
2.3.1.298 expressed in HeLa cells Mus musculus
2.3.1.298 expressed in HeLa cells Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
2.3.1.298 an ultra-long-chain fatty acyl-CoA + a sphingoid base Mus musculus C26-C36 an ultra-long-chain ceramide + CoA
-
 ?
2.3.1.298 an ultra-long-chain fatty acyl-CoA + a sphingoid base Homo sapiens C26-C36 an ultra-long-chain ceramide + CoA
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
2.3.1.298 Homo sapiens
-
-
-
2.3.1.298 Mus musculus
-
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
2.3.1.298 keratinocyte
-
 Mus musculus
-
2.3.1.298 keratinocyte
-
 Homo sapiens
-
2.3.1.298 skin the enzyme localizes to upper stratum spinosum and stratum granulosum Mus musculus
-
2.3.1.298 skin the enzyme localizes to upper stratum spinosum and stratum granulosum Homo sapiens
-
2.3.1.298 testis
-
 Mus musculus
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
2.3.1.298 an ultra-long-chain fatty acyl-CoA + a sphingoid base C26-C36 Mus musculus an ultra-long-chain ceramide + CoA
-
 ?
2.3.1.298 an ultra-long-chain fatty acyl-CoA + a sphingoid base C26-C36 Homo sapiens an ultra-long-chain ceramide + CoA
-
 ?
2.3.1.298 cerotoyl-CoA + sphingosine
-
 Mus musculus cerotoyl ceramide + CoA
-
 ?
2.3.1.298 cerotoyl-CoA + sphingosine
-
 Homo sapiens cerotoyl ceramide + CoA
-
 ?
2.3.1.298 lignoceroyl-CoA + sphingosine
-
 Mus musculus lignoceroyl ceramide + CoA
-
 ?
2.3.1.298 lignoceroyl-CoA + sphingosine
-
 Homo sapiens lignoceroyl ceramide + CoA
-
 ?
2.3.1.298 montanoyl-CoA + sphingosine bona fide substrate Mus musculus montanoyl ceramide + CoA
-
 ?
2.3.1.298 montanoyl-CoA + sphingosine bona fide substrate Homo sapiens montanoyl ceramide + CoA
-
 ?
2.3.1.298 additional information the enzyme also uses long-chain and very-long-chain fatty acyl-CoAs as substrate Mus musculus ?
-
 ?
2.3.1.298 additional information the enzyme also uses long-chain and very-long-chain fatty acyl-CoAs as substrate Homo sapiens ?
-
 ?
2.3.1.298 palmitoyl-CoA + sphingosine
-
 Mus musculus CoA + N-palmitoylsphingosine
-
 ?
2.3.1.298 palmitoyl-CoA + sphingosine
-
 Homo sapiens CoA + N-palmitoylsphingosine
-
 ?
2.3.1.298 stearoyl-CoA + sphingosine
-
 Mus musculus CoA + N-stearoylsphingosine
-
 ?
2.3.1.298 stearoyl-CoA + sphingosine
-
 Homo sapiens CoA + N-stearoylsphingosine
-
 ?

Synonyms

EC Number Synonyms Comment Organism
2.3.1.298 ceramide synthase 3
-
 Mus musculus
2.3.1.298 ceramide synthase 3
-
 Homo sapiens
2.3.1.298 CerS3
-
 Mus musculus
2.3.1.298 CerS3
-
 Homo sapiens

General Information

EC Number General Information Comment Organism
2.3.1.298 malfunction enzyme deficiency in mice results in complete loss of ultra-long-chain acyl moities (C26 or higher), lack of continuous extracellular lipid lamellae and a non-functional cornified lipid envelope. Consequently newborn mutant mice die shortly after birth from transepidermal water loss. Mutant skin is also prone to Candida albicans infection Mus musculus