Reactome: A Curated Pathway Database
Results 1 to 10 of 11
Pathways (5) Reactions (4) Proteins (1) Others (1)
Protein: UniProt:Q93063 EXT2 (Homo sapiens)
Last changed: 2015-03-10 08:59:22

Pathway: Metabolism (Homo sapiens)
Metabolic processes in human cells generate energy through the oxidation of molecules consumed in the diet and mediate the synthesis of diverse essential molecules not taken in the diet as well as the inactivation and elimination of toxic ones generated endogenously or present in the extracellular environment. The processes of energy metabolism can be classified into two groups according to whether the
Last changed: 2015-03-06 23:15:47

Pathway: Metabolism of carbohydrates (Homo sapiens)
These pathways together are responsible for: 1) the extraction of energy and carbon skeletons for biosyntheses from dietary sugars and related molecules; 2) the short-term storage of glucose in the body (as glycogen) and its mobilization during a short fast; and 3) the synthesis of glucose from pyruvate during extended fasts
Last changed: 2015-03-06 23:15:47

Pathway: Glycosaminoglycan metabolism (Homo sapiens)
Glycosaminoglycans (GAGs) are long, unbranched polysaccharides containing a repeating disaccharide unit composed of a hexosamine (either N-acetylgalactosamine (GalNAc) or N-acetylglucosamine (GlcNAc)) and a uronic acid (glucuronate or iduronate). They can be heavily sulfated. GAGs are located primarily in the extracellular matrix (ECM) and on cell membranes, acting as a lubricating fluid for joints and
Last changed: 2015-03-06 23:15:47

Pathway: Heparan sulfate/heparin (HS-GAG) metabolism (Homo sapiens)
The acronym HS-GAG is used to describe both heparin and heparan sulfate. HS-GAG is a member of the glycosaminoglycan family and consists of a variably sulfated repeating disaccharide unit, the most common one (50% of the total) being glucuronic acid (GlcA) linked to N-acetylglucosamine (GlcNAc). GlcA can be epimerized to iduronic acid. Higher degrees of sulfation and iduronic acid content in the polys
Last changed: 2015-03-06 23:15:47

Pathway: HS-GAG biosynthesis (Homo sapiens)
Heparan sulfate (HS) and heparin (sometimes collectively called HS-GAG) consist of the disaccharide unit GlcNAc-GlcA (N-acetylglucosamine-glucuronic acid) connected by a beta1,4 linkage. Heparin is exclusively made in mast cells whereas HS is made by virtually every type of cell in the body. As the chain length increases, the polysaccharides can undergo modifcations such as epimerisation of glucuronic
Last changed: 2015-03-06 23:15:47

Reaction: EXT1:EXT2 transfers GlcNAc to heparan (Homo sapiens)
Exostosin 1 and 2 (EXT1 and 2) are dual specificity enzymes which catalyze the addition of N-acetylglucosamine (GlcNAc) and glucuronate (GlcA) to the GAG protein linker sequence. The first addition mediated by these enzymes is that of glucuronate after EXTL1 has added the first GlcNAc unit to the linkage sequence. EXT1 and 2 form a heterodimer which translocates to the Golgi apparatus from the ER membr
Last changed: 2015-03-06 10:40:16

Reaction: EXT1:EXT2 transfers GlcA to heparan (Homo sapiens)
Exostosin 1 and 2 (EXT1 and 2) are dual specificity enzymes which catalyze the addition of N-acetylglucosamine (GlcNAc) and glucuronate (GlcA) to the GAG protein linker sequence. The first addition mediated by these enzymes is that of glucuronate after EXTL1 has added the first GlcNAc unit to the linkage sequence. EXT1 and 2 form a heterodimer which translocates to the Golgi apparatus from the ER membr
Last changed: 2015-03-06 10:40:16

Reaction: EXT1:EXT2 transfers GlcNAc to the terminal GlcA residue (Homo sapiens)
Exostosin1 and 2 (EXT1 and 2) are dual specificity enzymes which catalyze the addition of N acetylglucosamine (GlcNAc) and glucuronate (GlcA) to extend the GAG chain on the protein linker sequence. Heparan is synthesized once GlcNAc is transferred to this sequence. EXT1 and 2 form a heterodimer which translocates to the Golgi apparatus from the ER membrane (McCormick et al. 2000). Defects in EXT1 or 2
Last changed: 2015-03-06 10:40:16

Reaction: EXT1:EXT2 transfer GlcNAc to the heparan chain (Homo sapiens)
Exostosin1 and 2 (EXT1 and 2) are dual specificity enzymes which catalyze the addition of N acetylglucosamine (GlcNAc) and glucuronate (GlcA) to extend the GAG chain on the protein linker sequence. Heparan is synthesized once GlcNAc is transferred to this sequence. EXT1 and 2 form a heterodimer which translocates to the Golgi apparatus from the ER membrane (McCormick et al. 2000). Defects in EXT1 or 2
Last changed: 2015-03-06 10:40:16

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