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LARGE xylosyl- and glucuronyltransferase

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Bifunctional glycosyltransferase with both xylosyltransferase and beta-1,3-glucuronyltransferase activities involved in the biosynthesis of the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alpha-dystroglycan (DAG1) .

Below are the list of possible LARGE xylosyl- and glucuronyltransferase products. If you cannot find the target and/or product is not available in our catalog, please click here to contact us and request the product or submit your request for custom elisa kit production, custom recombinant protein production or custom antibody production. Custom ELISA Kits, Recombinant Proteins and Antibodies can be designed, manufactured and produced according to the researcher's specifications.
 

LARGE xylosyl- and glucuronyltransferase 1

 LARGE xylosyl- and glucuronyltransferase 1 ELISA Kit
 LARGE xylosyl- and glucuronyltransferase 1 Recombinant
 LARGE xylosyl- and glucuronyltransferase 1 Antibody
Also known as LARGE xylosyl- and glucuronyltransferase 1 (Acetylglucosaminyltransferase-like 1A) (Glycosyltransferase-like protein).
LARGE: Glycosyltransferase which participates in glycosylation of alpha-dystroglycan. May carry out the synthesis of glycoprotein and glycosphingolipid sugar chains. May be involved in the addition of a repeated disaccharide unit. Defects in LARGE are the cause of muscular dystrophy- dystroglycanopathy congenital with mental retardation type B6 (MDDGB6). A congenital muscular dystrophy associated with profound mental retardation, white matter changes and struc
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tural brain abnormalities. Skeletal muscle biopsies show reduced immunolabeling of alpha-dystroglycan. Defects in LARGE are the cause of muscular dystrophy- dystroglycanopathy congenital with brain and eye anomalies type A6 (MDDGA6); also called muscle-eye-brain disease LARGE- related or Walker-Warburg syndrome LARGE-related. MDDGA6 is an autosomal recessive disorder characterized by congenital muscular dystrophy associated with cobblestone lissencephaly and other brain anomalies, eye malformations, profound mental retardation, and death usually in the first years of life. Included diseases are the more severe Walker-Warburg syndrome and the slightly less severe muscle-eye-brain disease. Belongs to the glycosyltransferase 8 family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 2.4.-.-; Membrane protein, integral; Transferase

Chromosomal Location of Human Ortholog: 22q12.3

Cellular Component: Golgi apparatus; Golgi membrane; integral to Golgi membrane

Molecular Function: acetylglucosaminyltransferase activity; glucuronosyltransferase activity; manganese ion binding; transferase activity, transferring glycosyl groups; UDP-xylosyltransferase activity; xylosyltransferase activity

Biological Process: glycoprotein biosynthetic process; glycosphingolipid biosynthetic process; muscle maintenance; N-acetylglucosamine metabolic process; protein amino acid glycosylation; protein amino acid O-linked glycosylation; protein amino acid O-linked mannosylation; skeletal muscle regeneration

Disease: Muscular Dystrophy-dystroglycanopathy (congenital With Brain And Eye Anomalies), Type A, 1; Muscular Dystrophy-dystroglycanopathy (congenital With Brain And Eye Anomalies), Type A, 6; Muscular Dystrophy-dystroglycanopathy (congenital With Mental Retardation), Type B, 6
 LARGE1 ELISA Kit
 LARGE1 Recombinant
 LARGE1 Antibody
 KIAA0609 ELISA Kit
 KIAA0609 Recombinant
 KIAA0609 Antibody
 LARGE ELISA Kit
 LARGE Recombinant
 LARGE Antibody
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LARGE xylosyl- and glucuronyltransferase 2

 LARGE xylosyl- and glucuronyltransferase 2 ELISA Kit
 LARGE xylosyl- and glucuronyltransferase 2 Recombinant
 LARGE xylosyl- and glucuronyltransferase 2 Antibody
Also known as LARGE xylosyl- and glucuronyltransferase 2 (Glycosyltransferase-like 1B).
Bifunctional glycosyltransferase with both xylosyltransferase and beta-1,3-glucuronyltransferase activities involved in the biosynthesis of the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alpha-dystroglycan (DAG1). Phosphorylated O-mannosyl trisaccharid is required for binding laminin G-like domain-containing extracellular proteins with high affinity. Elongates the glucuronyl-beta-1,4-xylose-b
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eta disaccharide primer structure by adding repeating units [-3-Xylose-alpha-1,3-GlcA-beta-1-] to produce a heteropolysaccharide. Has a higher activity toward alpha-dystroglycan than LARGE.
 large2 ELISA Kit
 large2 Recombinant
 large2 Antibody
 gyltl1b ELISA Kit
 gyltl1b Recombinant
 gyltl1b Antibody
 si:ch211-206g24.1 ELISA Kit
 si:ch211-206g24.1 Recombinant
 si:ch211-206g24.1 Antibody
 si:ch211-282n12.1 ELISA Kit
 si:ch211-282n12.1 Recombinant
 si:ch211-282n12.1 Antibody
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LARGE xylosyl- and glucuronyltransferase 2-A

 LARGE xylosyl- and glucuronyltransferase 2-A ELISA Kit
 LARGE xylosyl- and glucuronyltransferase 2-A Recombinant
 LARGE xylosyl- and glucuronyltransferase 2-A Antibody
Also known as LARGE xylosyl- and glucuronyltransferase 2-A (Glycosyltransferase-like 1B-A).
Bifunctional glycosyltransferase with both xylosyltransferase and beta-1,3-glucuronyltransferase activities involved in the biosynthesis of the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alph
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a-dystroglycan (DAG1). Phosphorylated O-mannosyl trisaccharid is required for binding laminin G-like domain-containing extracellular proteins with high affinity. Elongates the glucuronyl-beta-1,4-xylose-beta disaccharide primer structure by adding repeating units [-3-Xylose-alpha-1,3-GlcA-beta-1-] to produce a heteropolysaccharide. Has a higher activity toward alpha-dystroglycan than LARGE.
 large2-a ELISA Kit
 large2-a Recombinant
 large2-a Antibody
 gyltl1b-a ELISA Kit
 gyltl1b-a Recombinant
 gyltl1b-a Antibody
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LARGE xylosyl- and glucuronyltransferase 2-B

 LARGE xylosyl- and glucuronyltransferase 2-B ELISA Kit
 LARGE xylosyl- and glucuronyltransferase 2-B Recombinant
 LARGE xylosyl- and glucuronyltransferase 2-B Antibody
Also known as LARGE xylosyl- and glucuronyltransferase 2-B (Glycosyltransferase-like 1B-B).
Bifunctional glycosyltransferase with both xylosyltransferase and beta-1,3-glucuronyltransferase activities involved in the biosynthesis of the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alph
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a-dystroglycan (DAG1). Phosphorylated O-mannosyl trisaccharid is required for binding laminin G-like domain-containing extracellular proteins with high affinity. Elongates the glucuronyl-beta-1,4-xylose-beta disaccharide primer structure by adding repeating units [-3-Xylose-alpha-1,3-GlcA-beta-1-] to produce a heteropolysaccharide. Has a higher activity toward alpha-dystroglycan than LARGE.
 large2-b ELISA Kit
 large2-b Recombinant
 large2-b Antibody
 gyltl1b-b ELISA Kit
 gyltl1b-b Recombinant
 gyltl1b-b Antibody
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