Showing metabocard for Ceramide (d18:1/18:0) (BMDB0004950)

General function:

Involved in ceramidase activity

Specific function:

Lysosomal ceramidase that hydrolyzes sphingolipid ceramides into sphingosine and free fatty acids at acidic pH (By similarity). Ceramides, sphingosine, and its phosphorylated form sphingosine-1-phosphate are bioactive lipids that mediate cellular signaling pathways regulating several biological processes including cell proliferation, apoptosis and differentiation (By similarity). Has a higher catalytic efficiency towards C12-ceramides versus other ceramides (By similarity). Also catalyzes the reverse reaction allowing the synthesis of ceramides from fatty acids and sphingosine (By similarity). For the reverse synthetic reaction, the natural sphingosine D-erythro isomer is more efficiently utilized as a substrate compared to D-erythro-dihydrosphingosine and D-erythro-phytosphingosine, while the fatty acids with chain lengths of 12 or 14 carbons are the most efficiently used (By similarity). Has also an N-acylethanolamine hydrolase activity (By similarity). By regulating the levels of ceramides, sphingosine and sphingosine-1-phosphate in the epidermis, mediates the calcium-induced differentiation of epidermal keratinocytes (By similarity). Also indirectly regulates tumor necrosis factor/TNF-induced apoptosis (By similarity). By regulating the intracellular balance between ceramides and sphingosine, in adrenocortical cells, probably also acts as a regulator of steroidogenesis (By similarity).

Gene Name:

ASAH1

Uniprot ID:

Q17QB3

Molecular weight:

44965.0

General function:

Involved in hydrolase activity, acting on glycosyl bonds

Specific function:

Converts sphingomyelin to ceramide. Also has phospholipase C activities toward 1,2-diacylglycerolphosphocholine and 1,2-diacylglycerolphosphoglycerol.

Gene Name:

SMPD1

Uniprot ID:

Q0VD19

Molecular weight:

69392.0

General function:

Involved in cation binding

Specific function:

Glucosylceramidase that catalyzes, within the lysosomal compartment, the hydrolysis of glucosylceramide/GlcCer into free ceramide and glucose. Thereby, plays a central role in the degradation of complex lipids and the turnover of cellular membranes. Through the production of ceramides, participates to the PKC-activated salvage pathway of ceramide formation. Also plays a role in cholesterol metabolism. May either catalyze the glucosylation of cholesterol, through a transglucosylation reaction that transfers glucose from glucosylceramide to cholesterol. The short chain saturated C8:0-GlcCer and the mono-unsaturated C18:0-GlcCer being the most effective glucose donors for that transglucosylation reaction. Under specific conditions, may alternatively catalyze the reverse reaction, transferring glucose from cholesteryl-beta-D-glucoside to ceramide. Finally, may also hydrolyze cholesteryl-beta-D-glucoside to produce D-glucose and cholesterol.

Gene Name:

GBA

Uniprot ID:

Q2KHZ8

Molecular weight:

59855.0

General function:

Carbohydrate transport and metabolism

Specific function:

UDP-glucuronosyltransferases catalyze phase II biotransformation reactions in which lipophilic substrates are conjugated with glucuronic acid to increase water solubility and enhance excretion. They are of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds (By similarity).

Gene Name:

UGT3A1

Uniprot ID:

Q1LZI1

Molecular weight:

59682.0

Reactions

General function:

Involved in lipid transport

Specific function:

Shelters ceramides and diacylglycerol lipids inside its START domain and mediates the intracellular trafficking of ceramides and diacylglycerol lipids in a non-vesicular manner.

Gene Name:

CERT1

Uniprot ID:

Q9GKI7

Molecular weight:

70847.0

General function:

Not Available

Specific function:

Magnesium-independent phospholipid phosphatase that catalyzes the dephosphorylation of a variety of glycerolipid and sphingolipid phosphate esters including phosphatidate/PA, lysophosphatidate/LPA, sphingosine 1-phosphate/S1P and ceramide 1-phosphate/C1P. Has no apparent extracellular phosphatase activity and therefore most probably acts intracellularly. Also acts on N-oleoyl ethanolamine phosphate/N-(9Z-octadecenoyl)-ethanolamine phosphate, a potential physiological compound. Through dephosphorylation of these bioactive lipid mediators produces new bioactive compounds and may regulate signal transduction in different cellular processes (By similarity). Indirectly regulates, for instance, cell cycle G1/S phase transition through its phospholipid phosphatase activity (By similarity).

Gene Name:

PLPP2

Uniprot ID:

Q2HJ61

Molecular weight:

32328.0

Reactions

General function:

Not Available

Specific function:

Not Available

Gene Name:

SGPP2

Uniprot ID:

G3MXX9

Molecular weight:

44782.0

Reactions

General function:

Not Available

Specific function:

Bifunctional enzyme which acts as both a sphingolipid delta(4)-desaturase and a sphingolipid C4-monooxygenase.

Gene Name:

DEGS2

Uniprot ID:

Q0II71

Molecular weight:

37319.0

Reactions

General function:

Not Available

Specific function:

Not Available

Gene Name:

CERK

Uniprot ID:

G3MXL8

Molecular weight:

60069.0

Reactions

General function:

Not Available

Specific function:

Not Available

Gene Name:

GALC

Uniprot ID:

E1B7K0

Molecular weight:

77833.0

Reactions