LARGE - Glycosyltransferase-like protein LARGE1 - human protein (Function)
 
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LARGE »  Glycosyltransferase-like protein LARGE1   [ EC 2.4.-.- ]
 
Protein also known as:  Acetylglucosaminyltransferase-like 1A.
Gene name:  LARGE
Family names: In the N-terminal section Glycosyltransferase 8
In the C-terminal section Glycosyltransferase 49
Entry whose protein(s) existence is based on evidence at protein level
extend overview
1 45 2
GENE REF ISO

Function

 show evidences
Overview 
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) (PubMed22223806). Phosphorylated O-mannosyl trisaccharid is required for binding laminin G-like domain-containing extracellular proteins with high affinity and plays a key role in skeletal muscle function and regeneration. LARGE elongates the glucuronyl-beta-1,4-xylose-beta disaccharide primer structure initiated by B3GNT1/B4GAT1 by adding repeating units [-3-Xylose-alpha-1,3-GlcA-beta-1-] to produce a heteropolysaccharide (PubMed25279699).  
8
  • UniProtKB
GO molecular function 
Acetylglucosaminyltransferase activitydefinition[GO:0008375]  
1
  • PINC
Glucuronosyltransferase activitydefinition[GO:0015020]  
3
  • UniProtKB
Manganese ion bindingdefinition[GO:0030145]  
1
  • UniProtKB
Transferase activity, transferring glycosyl groupsdefinition[GO:0016757]  
1
  • UniProtKB
Xylosyltransferase activitydefinition[GO:0042285]  
2
  • UniProtKB
GO biological process 
Glycoprotein biosynthetic processdefinition[GO:0009101]  
1
  • UniProtKB
Glycosphingolipid biosynthetic processdefinition[GO:0006688]  
1
  • UniProtKB
Muscle cell cellular homeostasisdefinition[GO:0046716]  
1
  • Ortholog Curator
N-acetylglucosamine metabolic processdefinition[GO:0006044]  
1
  • PINC
Protein glycosylationdefinition[GO:0006486]  
1
  • PINC
Protein O-linked mannosylationdefinition[GO:0035269]  
4
  • UniProtKB
Skeletal muscle organ developmentdefinition[GO:0060538]  
1
  • Ortholog Curator
Skeletal muscle tissue regenerationdefinition[GO:0043403]  
1
  • Ortholog Curator
Enzymatic activity 
It requires the following cofactor
 Mn(2+) : Binds 2 Mn(2+) ions per subunit. The xylosyltransferase part binds one Mn(2+) and the beta-1,3-glucuronyltransferase part binds one Mn(2+).  
2
  • UniProtKB
According to CAZy, this protein is belongs to the following pathways:
Glycosyltransferase Family 49  GT49  
Glycosyltransferase Family 8  GT8  
Pathways 
This protein is involved in the following pathway
Protein modification; protein glycosylation  
3
  • UniProtKB
 

Keywords

Molecular function 
Glycosyltransferase  definition   [KW-0328]
Transferase  definition   [KW-0808]
Technical term 
Multifunctional enzyme  definition   [KW-0511]
Reference proteome  definition   [KW-1185]
 

Further external links

GeneWiki: LARGE
GenomeRNAi: 9215
PRO: PR:O95461