Showing metabocard for Glucosylceramide (HMDB00140)

General function:

Involved in hydrolase activity, hydrolyzing O-glycosyl compounds

Specific function:

Cleaves beta-linked terminal galactosyl residues from gangliosides, glycoproteins, and glycosaminoglycans. Isoform 2 has no beta-galactosidase catalytic activity, but plays functional roles in the formation of extracellular elastic fibers (elastogenesis) and in the development of connective tissue. Seems to be identical to the elastin-binding protein (EBP), a major component of the non-integrin cell surface receptor expressed on fibroblasts, smooth muscle cells, chondroblasts, leukocytes, and certain cancer cell types. In elastin producing cells, associates with tropoelastin intracellularly and functions as a recycling molecular chaperone which facilitates the secretions of tropoelastin and its assembly into elastic fibers.

Gene Name:

GLB1

Uniprot ID:

P16278

Molecular weight:

Not Available

General function:

Cell wall/membrane/envelope biogenesis

Specific function:

Catalyzes the first glycosylation step in glycosphingolipid biosynthesis, the transfer of glucose to ceramide. May also serve as a "flippase".

Gene Name:

UGCG

Uniprot ID:

Q16739

Molecular weight:

44853.255

General function:

Involved in hydrolase activity, hydrolyzing O-glycosyl compounds

Specific function:

LPH splits lactose in the small intestine.

Gene Name:

LCT

Uniprot ID:

P09848

Molecular weight:

218584.77

General function:

Involved in catalytic activity

Specific function:

Not Available

Gene Name:

GBA

Uniprot ID:

P04062

Molecular weight:

59715.745

General function:

Involved in transferase activity, transferring glycosyl groups

Specific function:

Required for the biosynthesis of glycosphingolipids.

Gene Name:

B4GALT6

Uniprot ID:

Q9UBX8

Molecular weight:

44913.315

Reactions

General function:

Involved in cysteine-type endopeptidase activity

Specific function:

Mediates GPI anchoring in the endoplasmic reticulum, by replacing a protein's C-terminal GPI attachment signal peptide with a pre-assembled GPI. During this transamidation reaction, the GPI transamidase forms a carbonyl intermediate with the substrate protein

Gene Name:

PIGK

Uniprot ID:

Q92643

Molecular weight:

45251.4

General function:

Involved in biosynthetic process

Specific function:

Necessary for the synthesis of N-acetylglucosaminyl-phosphatidylinositol, the very early intermediate in GPI-anchor biosynthesis.

Gene Name:

PIGA

Uniprot ID:

P37287

Molecular weight:

54126.065

General function:

Involved in catalytic activity

Specific function:

Non-lysosomal glucosylceramidase that catalyzes the conversion of glucosylceramide to free glucose and ceramide. Involved in sphingomyelin generation and prevention of glycolipid accumulation. May also catalyze the hydrolysis of bile acid 3-O-glucosides, however, the relevance of such activity is unclear in vivo.

Gene Name:

GBA2

Uniprot ID:

Q9HCG7

Molecular weight:

104648.13

General function:

Involved in GPI anchor biosynthetic process

Specific function:

Component of the GPI transamidase complex. May be involved in the recognition of either the GPI attachment signal or the lipid portion of GPI

Gene Name:

PIGU

Uniprot ID:

Q9H490

Molecular weight:

50051.2

General function:

Involved in protein binding

Specific function:

Component of the GPI transamidase complex. Essential for transfer of GPI to proteins, particularly for formation of carbonyl intermediates

Gene Name:

PIGT

Uniprot ID:

Q969N2

Molecular weight:

65699.0

General function:

Involved in transferase activity, transferring hexosyl groups

Specific function:

Catalyzes the formation of some glycolipid via the addition of N-acetylgalactosamine (GalNAc) in alpha-1,3-linkage to some substrate. Glycolipids probably serve for adherence of some pathogens

Gene Name:

GBGT1

Uniprot ID:

Q8N5D6

Molecular weight:

40126.9

General function:

Involved in transferase activity, transferring hexosyl groups

Specific function:

Mannosyltransferase involved in glycosylphosphatidylinositol-anchor biosynthesis. Transfers the first alpha-1,4-mannose to GlcN-acyl-PI during GPI precursor assembly

Gene Name:

PIGM

Uniprot ID:

Q9H3S5

Molecular weight:

49459.2

General function:

Involved in protein binding

Specific function:

Component of the GPI transamidase complex. Essential for transfer of GPI to proteins, particularly for formation of carbonyl intermediates

Gene Name:

PIGS

Uniprot ID:

Q96S52

Molecular weight:

61655.5

General function:

Involved in galactosyltransferase activity

Specific function:

Beta-1,3-N-acetylglucosaminyltransferase that plays a key role in the synthesis of lacto- or neolacto-series carbohydrate chains on glycolipids, notably by participating in biosynthesis of HNK-1 and Lewis X carbohydrate structures. Has strong activity toward lactosylceramide (LacCer) and neolactotetraosylceramide (nLc(4)Cer; paragloboside), resulting in the synthesis of Lc(3)Cer and neolactopentaosylceramide (nLc(5)Cer), respectively. Probably plays a central role in regulating neolacto-series glycolipid synthesis during embryonic development.

Gene Name:

B3GNT5

Uniprot ID:

Q9BYG0

Molecular weight:

44052.295

General function:

Involved in transferase activity, transferring glycosyl groups

Specific function:

Mannosyltransferase involved in glycosylphosphatidylinositol-anchor biosynthesis. Transfers a fourth mannose to some trimannosyl-GPIs during GPI precursor assembly. The presence of a fourth mannose in GPI is facultative and only scarcely detected, suggesting that it only exists in some tissues

Gene Name:

PIGZ

Uniprot ID:

Q86VD9

Molecular weight:

63472.6

General function:

Involved in phosphatidylinositol N-acetylglucosaminyltransferase activity

Specific function:

Part of the complex catalyzing the transfer of N-acetylglucosamine from UDP-N-acetylglucosamine to phosphatidylinositol, the first step of GPI biosynthesis.

Gene Name:

PIGQ

Uniprot ID:

Q9BRB3

Molecular weight:

65343.25

General function:

Involved in transferase activity, transferring acyl groups

Specific function:

Probable acetyltransferase, which acetylates the inositol ring of phosphatidylinositol during biosynthesis of GPI-anchor. Acetylation during GPI-anchor biosynthesis is not essential for the subsequent mannosylation and is usually removed soon after the attachment of GPIs to proteins (By similarity).

Gene Name:

PIGW

Uniprot ID:

Q7Z7B1

Molecular weight:

Not Available

General function:

Involved in transferase activity, transferring glycosyl groups

Specific function:

Mannosyltransferase involved in glycosylphosphatidylinositol-anchor biosynthesis. Transfers the third alpha-1,2-mannose to Man2-GlcN-acyl-PI during GPI precursor assembly

Gene Name:

PIGB

Uniprot ID:

Q92521

Molecular weight:

65055.9

General function:

Involved in catalytic activity

Specific function:

Ethanolamine phosphate transferase involved in glycosylphosphatidylinositol-anchor biosynthesis. Transfers ethanolamine phosphate to the first alpha-1,4-linked mannose of the glycosylphosphatidylinositol precursor of GPI-anchor

Gene Name:

PIGN

Uniprot ID:

O95427

Molecular weight:

105809.2

General function:

Involved in catalytic activity

Specific function:

Ethanolamine phosphate transferase involved in glycosylphosphatidylinositol-anchor biosynthesis. Transfers ethanolamine phosphate to the GPI third mannose which links the GPI-anchor to the C-terminus of the proteins by an amide bond

Gene Name:

PIGO

Uniprot ID:

Q8TEQ8

Molecular weight:

118697.6

General function:

Involved in GPI anchor biosynthetic process

Specific function:

Component of the GPI-GlcNAc transferase (GPI-GnT) complex in the endoplasmic reticulum, a complex that catalyzes transfer of GlcNAc from UDP-GlcNAc to an acceptor phosphatidylinositol, the first step in the production of GPI- anchors for cell surface proteins. May act by regulating the catalytic subunit PIGA

Gene Name:

PIGY

Uniprot ID:

Q3MUY2

Molecular weight:

8057.5

General function:

Involved in phosphatidylinositol N-acetylglucosaminyltransferase activity

Specific function:

Part of the complex catalyzing the transfer of N-acetylglucosamine from UDP-N-acetylglucosamine to phosphatidylinositol, the first step of GPI biosynthesis.

Gene Name:

PIGC

Uniprot ID:

Q92535

Molecular weight:

33582.18

General function:

Involved in phosphatidylinositol N-acetylglucosaminyltr

Specific function:

Part of the complex catalyzing the transfer of N-acetylglucosamine from UDP-N-acetylglucosamine to phosphatidylinositol, the first step of GPI biosynthesis.

Gene Name:

PIGP

Uniprot ID:

P57054

Molecular weight:

18089.055

General function:

Involved in transferase activity, transferring hexosyl groups

Specific function:

Alpha-1,6-mannosyltransferase involved in glycosylphosphatidylinositol-anchor biosynthesis. Transfers the second mannose to the glycosylphosphatidylinositol during GPI precursor assembly

Gene Name:

PIGV

Uniprot ID:

Q9NUD9

Molecular weight:

55712.1

General function:

Involved in galactosyltransferase activity

Specific function:

Catalyzes the transfer of Gal to GlcNAc-based acceptors with a preference for the core3 O-linked glycan GlcNAc(beta1,3)GalNAc structure. Can use glycolipid LC3Cer as an efficient acceptor

Gene Name:

B3GALT5

Uniprot ID:

Q9Y2C3

Molecular weight:

36188.9

General function:

Involved in cholesterol binding

Specific function:

May be involved in the regulation of the lipid composition of sperm membranes during the maturation in the epididymis

Gene Name:

NPC2

Uniprot ID:

P61916

Molecular weight:

16570.1

General function:

Involved in catalytic activity

Specific function:

Ethanolamine phosphate transferase involved in glycosylphosphatidylinositol-anchor biosynthesis. Transfers ethanolamine phosphate to the GPI second mannose

Gene Name:

PIGG

Uniprot ID:

Q5H8A4

Molecular weight:

108171.7

General function:

Involved in phosphatidylinositol N-acetylglucosaminyltr

Specific function:

Part of the complex catalyzing the transfer of N-acetylglucosamine from UDP-N-acetylglucosamine to phosphatidylinositol, the first step of GPI biosynthesis.

Gene Name:

PIGH

Uniprot ID:

Q14442

Molecular weight:

21080.415

General function:

Involved in N-acetylglucosaminylphosphatidylinositol de

Specific function:

Involved in the second step of GPI biosynthesis. De-N-acetylation of N-acetylglucosaminyl-phosphatidylinositol.

Gene Name:

PIGL

Uniprot ID:

Q9Y2B2

Molecular weight:

28530.965

General function:

Involved in metal ion binding

Specific function:

Converts sphingomyelin to ceramide. Hydrolyze 1-acyl-2-lyso-sn-glycero-3-phosphocholine (lyso-PC) and 1-O-alkyl-2-lyso-sn-glycero-3-phosphocholine (lyso-platelet-activating factor). The physiological substrate seems to be Lyso-PAF.

Gene Name:

SMPD2

Uniprot ID:

O60906

Molecular weight:

47645.29

General function:

Involved in galactosylceramidase activity

Specific function:

Hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. Enzyme with very low activity responsible for the lysosomal catabolism of galactosylceramide, a major lipid in myelin, kidney and epithelial cells of small intestine and colon.

Gene Name:

GALC

Uniprot ID:

P54803

Molecular weight:

77062.86

General function:

Involved in catalytic activity

Specific function:

Converts sphingomyelin to ceramide. Also has phospholipase C activity toward palmitoyl lyso-phosphocholine. Does not appear to have nucleotide pyrophosphatase activity.

Gene Name:

ENPP7

Uniprot ID:

Q6UWV6

Molecular weight:

51493.415

General function:

Involved in hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds, in linear amides

Specific function:

Hydrolyzes the sphingolipid ceramide into sphingosine and free fatty acid at an optimal pH of 8.0. Has a highly restricted substrate specificity for the natural stereoisomer of ceramide with D-erythro-sphingosine but not D-ribo-phytosphingosine or D-erythro-dihydrosphingosine as a backbone. May have a role in regulating the levels of bioactive lipids ceramide and sphingosine 1-phosphate, as well as complex sphingolipids (By similarity).

Gene Name:

ACER1

Uniprot ID:

Q8TDN7

Molecular weight:

31095.165

General function:

Involved in hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds, in linear amides

Specific function:

Hydrolyzes the sphingolipid ceramide into sphingosine and free fatty acid. Unsaturated long-chain ceramides are the best substrates, saturated long-chain ceramides and unsaturated very long-chain ceramides are good substrates, whereas saturated very long-chain ceramides and short-chain ceramides were poor substrates. The substrate preference is D-erythro-C(18:1)-, C(20:1)-, C(20:4)-ceramide > D-erythro-C(16:0)-, C(18:0), C(20:0)-ceramide > D-erythro-C(24:1)-ceramide > D-erythro-C(12:0)-ceramide, D-erythro-C(14:0)-ceramides > D-erythro-C(24:0)-ceramide > D-erythro-C(6:0)-ceramide. Inhibits the maturation of protein glycosylation in the Golgi complex, including that of integrin beta-1 (ITGB1) and of LAMP1, by increasing the levels of sphingosine. Inhibits cell adhesion by reducing the level of ITGB1 in the cell surface. May have a role in cell proliferation and apoptosis that seems to depend on the balance between sphingosine and sphingosine-1-phosphate.

Gene Name:

ACER2

Uniprot ID:

Q5QJU3

Molecular weight:

31308.85

General function:

Involved in metal ion binding

Specific function:

Catalyzes the hydrolysis of sphingomyelin to form ceramide and phosphocholine. Ceramide mediates numerous cellular functions, such as apoptosis and growth arrest, and is capable of regulating these 2 cellular events independently. Also hydrolyzes sphingosylphosphocholine. Regulates the cell cycle by acting as a growth suppressor in confluent cells. Probably acts as a regulator of postnatal development and participates in bone and dentin mineralization.

Gene Name:

SMPD3

Uniprot ID:

Q9NY59

Molecular weight:

71080.1

General function:

Involved in metal ion binding

Specific function:

Catalyzes the hydrolysis of membrane sphingomyelin to form phosphorylcholine and ceramide.

Gene Name:

SMPD4

Uniprot ID:

Q9NXE4

Molecular weight:

86192.575

General function:

Involved in ceramidase activity

Specific function:

May hydrolyze the sphingolipid ceramide into sphingosine and free fatty acid (By similarity).

Gene Name:

ASAH2C

Uniprot ID:

P0C7U2

Molecular weight:

Not Available

General function:

Involved in lipid metabolic process

Specific function:

Hydrolyzes the sphingolipid ceramide into sphingosine and free fatty acid.

Gene Name:

ASAH1

Uniprot ID:

Q13510

Molecular weight:

44045.27

General function:

Involved in ceramidase activity

Specific function:

Hydrolyzes the sphingolipid ceramide into sphingosine and free fatty acid at an optimal pH of 6.5-8.5. Acts as a key regulator of sphingolipid signaling metabolites by generating sphingosine at the cell surface. Acts as a repressor of apoptosis both by reducing C16-ceramide, thereby preventing ceramide-induced apoptosis, and generating sphingosine, a precursor of the antiapoptotic factor sphingosine 1-phosphate. Probably involved in the digestion of dietary sphingolipids in intestine by acting as a key enzyme for the catabolism of dietary sphingolipids and regulating the levels of bioactive sphingolipid metabolites in the intestinal tract.

Gene Name:

ASAH2

Uniprot ID:

Q9NR71

Molecular weight:

19024.55

General function:

Involved in hydrolase activity

Specific function:

Converts sphingomyelin to ceramide. Also has phospholipase C activities toward 1,2-diacylglycerolphosphocholine and 1,2-diacylglycerolphosphoglycerol. Isoform 2 and isoform 3 have lost catalytic activity.

Gene Name:

SMPD1

Uniprot ID:

P17405

Molecular weight:

69935.53

General function:

Involved in tubulin binding

Specific function:

Essential for GPI-anchoring of precursor proteins but not for GPI synthesis. Acts before or during formation of the carbonyl intermediate

Gene Name:

GPAA1

Uniprot ID:

O43292

Molecular weight:

67622.5

General function:

Involved in GPI anchor biosynthetic process

Specific function:

Involved in GPI-anchor biosynthesis through the transfer of ethanolamine phosphate to the third mannose of GPI

Gene Name:

PIGF

Uniprot ID:

Q07326

Molecular weight:

24889.3

General function:

Involved in glycolipid transporter activity

Specific function:

Not Available

Gene Name:

PLEKHA9

Uniprot ID:

O95397

Molecular weight:

43538.3

General function:

Involved in glycolipid transporter activity

Specific function:

Not Available

Gene Name:

GLTPD2

Uniprot ID:

A6NH11

Molecular weight:

31641.2

General function:

Involved in glycolipid transporter activity

Specific function:

Not Available

Gene Name:

PLEKHA8

Uniprot ID:

B5MDU3

Molecular weight:

49308.8

General function:

Involved in immune response

Specific function:

Antigen-presenting protein that binds self and non-self lipid and glycolipid antigens and presents them to T-cell receptors on natural killer T-cells

Gene Name:

CD1A

Uniprot ID:

P06126

Molecular weight:

37077.1

General function:

Involved in GPI anchor biosynthetic process

Specific function:

Essential component of glycosylphosphatidylinositol- mannosyltransferase 1 which transfers the first of the 4 mannoses in the GPI-anchor precursors during GPI-anchor biosynthesis. Probably acts by stabilizing the mannosyltransferase PIGM

Gene Name:

PIGX

Uniprot ID:

Q8TBF5

Molecular weight:

28788.1

General function:

Involved in sphingolipid activator protein activity

Specific function:

Binds gangliosides and stimulates ganglioside GM2 degradation. It stimulates only the breakdown of ganglioside GM2 and glycolipid GA2 by beta-hexosaminidase A. It extracts single GM2 molecules from membranes and presents them in soluble form to beta-hexosaminidase A for cleavage of N-acetyl-D-galactosamine and conversion to GM3

Gene Name:

GM2A

Uniprot ID:

P17900

Molecular weight:

20838.1

General function:

Involved in glycolipid transporter activity

Specific function:

Not Available

Gene Name:

GLTPD1

Uniprot ID:

Q5TA50

Molecular weight:

24364.8

General function:

Involved in immune response

Specific function:

Antigen-presenting protein that binds self and non-self lipid and glycolipid antigens and presents them to T-cell receptors on natural killer T-cells

Gene Name:

CD1C

Uniprot ID:

P29017

Molecular weight:

37653.7