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LARGE1

Gene
large1
Protein
LARGE xylosyl- and glucuronyltransferase 1
Organism
Danio rerio
Length
757 amino acids
Function
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 and plays a key role in skeletal muscle function and regeneration. LARGE 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.
Similarity
In the N-terminal section; belongs to the glycosyltransferase 8 family.
Mass
87.673 kDa
Sequence
MLGMCRGRRKFVAASLALIFIPALTWLYLSSANITVKPLPLSPLDPQSSAVVGAAAEHQSLELRLRDVEEHNNALRREISRTPRVPTHSSHPSSSRHGNQLHTHSTEEGTGDSEAKKGAAAGNSSDCVPQPVVKKCETIHIAIVCAGYNASRDVVTLVKSVLFHRRNPLHFHIITDSIARKILADLFHTWMVPAVHVNFYDADELKSEVSWIPNKHYSGIHGLMKLVLTKTLPSDLEKVIVLDTDITFATDIAELWVVFHKFKGQQVLGLVENQSDWYLGNLWKNHRPWPALGRGFNTGVILLLLDRLRKLKWEQMWRLTAERELMSMLSTSLADQDIFNAVIKQNPFLVHQLPCYWNVQLSDHTRSEKCYKDVSDLKVIHWNSPKKLRVKNKHVEFFRNLYLTFLEYDGNLLRRELFGCPSETDHNSENLQKTLSELDEDDPCYEFRRERFTVHRTHVYFLHYEYEPTVDNTDVTLVAQLSMDRLQMLEAICKHWEGPISLALYLSDAEAQQFLRYAQGSEVLMSRSNVGYHIVYKEGQFYPVNLLRNVAMGQVNTPYMFLSDIDFLPMYGLYEYLRKSVVQLDMGNTKKALVVPAFETLRYRLSFPKSKAELLSQLDMGTLFTFRYHVWTKGHAPTDFAKWRTATTPYRVQWEADFEPYVMVRRESPEYDRRFVGFGWNKVAHIMELDAQEYEFVVLPNAYMIHMPHAPSFDITKFRSNKQYRACLKTLKEEFQQNMSRRYGFAALKYMTVDNNS

Gene
LARGE1
Protein
LARGE xylosyl- and glucuronyltransferase 1
Organism
Gallus gallus
Length
756 amino acids
Function
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 and plays a key role in skeletal muscle function and regeneration. LARGE 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.
Similarity
In the N-terminal section; belongs to the glycosyltransferase 8 family.
Mass
88.046 kDa
Sequence
MLGICRGRRKFLAASLTVLFVPAVTWIYLFAGSFEDGKPVSLSPLESQPHSPRYTASSQRDRESLEVRMREVEEENRVLRKQLSLAQGRSPSHHRGNHSKTYSMEEGTGDSESLRAGIVAGNSSECGQQPAVEKCETIHVAIVCAGYNASRDVVTLVKSVLFHRRNPLHFHLIADAIAKQILATLFQTWMVPAVRIDFYDADELKSEVSWIPNKHYSGIYGLMKLVLTKTLPSNLERVIVLDTDITFATDIAELWAVFHKFKGQQVLGLVENQSDWYLGNLWKNHRPWPALGRGYNTGVILLLLDKLRKMKWEQMWRLTAERELMSMLSTSLADQDIFNAVIKQNPFLVYQLPCFWNVQLSDHTRSEQCYRDVSDLKVIHWNSPKKLRVKNKHVEFFRNLYLTFLEYDGNLLRRELFGCPSEADVNSENLQKQLSEPDEDDLCYEFRRERFTVHRTHLYFLHYEYEPASDNTDVTLVAQLSMDRLQMLEAICKHWEDPISLALYLSDAEAQQFLRYAQGSEVLMSRHNVGYHIVYKEGQFYPVNLLRNVAMKHISTPYMFLSDIDFLPMYGLYEYLRKSVTQLDLANTKKALIIPAFETLRYRLSFPKSKAELLSMLDMGTLFTFRYHVWTKGHAPTNFAKWRTATTPYRVEWEADFEPYVVVRKDCPEYDRRFVGFGWNKVAHIMELDAQEYEFTVLPNAYMIHMPHAPSFDITKFRSNKQYRICLKTLKEEFQQDMSRHYGFAALKYLTAENNS

Gene
LARGE1
Protein
LARGE xylosyl- and glucuronyltransferase 1
Organism
Homo sapiens
Length
756 amino acids
Function
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) (PubMed:22223806). 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 (PubMed:25279699).
Similarity
In the N-terminal section; belongs to the glycosyltransferase 8 family.
Mass
88.066 kDa
Sequence
MLGICRGRRKFLAASLSLLCIPAITWIYLFSGSFEDGKPVSLSPLESQAHSPRYTASSQRERESLEVRMREVEEENRALRRQLSLAQGRAPSHRRGNHSKTYSMEEGTGDSENLRAGIVAGNSSECGQQPVVEKCETIHVAIVCAGYNASRDVVTLVKSVLFHRRNPLHFHLIADSIAEQILATLFQTWMVPAVRVDFYNADELKSEVSWIPNKHYSGIYGLMKLVLTKTLPANLERVIVLDTDITFATDIAELWAVFHKFKGQQVLGLVENQSDWYLGNLWKNHRPWPALGRGYNTGVILLLLDKLRKMKWEQMWRLTAERELMGMLSTSLADQDIFNAVIKQNPFLVYQLPCFWNVQLSDHTRSEQCYRDVSDLKVIHWNSPKKLRVKNKHVEFFRNLYLTFLEYDGNLLRRELFGCPSEADVNSENLQKQLSELDEDDLCYEFRRERFTVHRTHLYFLHYEYEPAADSTDVTLVAQLSMDRLQMLEAICKHWEGPISLALYLSDAEAQQFLRYAQGSEVLMSRHNVGYHIVYKEGQFYPVNLLRNVAMKHISTPYMFLSDIDFLPMYGLYEYLRKSVIQLDLANTKKAMIVPAFETLRYRLSFPKSKAELLSMLDMGTLFTFRYHVWTKGHAPTNFAKWRTATTPYRVEWEADFEPYVVVRRDCPEYDRRFVGFGWNKVAHIMELDVQEYEFIVLPNAYMIHMPHAPSFDITKFRSNKQYRICLKTLKEEFQQDMSRRYGFAALKYLTAENNS

Gene
Large1
Protein
LARGE xylosyl- and glucuronyltransferase 1
Organism
Mus musculus
Length
756 amino acids
Function
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) (PubMed:23125099, PubMed:23135544). 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 (PubMed:15184894, PubMed:24132234). 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 (By similarity).
Similarity
In the N-terminal section; belongs to the glycosyltransferase 8 family.
Mass
87.964 kDa
Sequence
MLGICRGRRKFLAASLTLLCIPAITWIYLFAGSFEDGKPVSLSPLESQAHSPRYTASSQRERESLEVRVREVEEENRALRRQLSLAQGQSPAHHRGNHSKTYSMEEGTGDSENLRAGIVAGNSSECGQQPAVEKCETIHVAIVCAGYNASRDVVTLVKSVLFHRRNPLHFHLIADSIAEQILATLFQTWMVPAVRVDFYNADELKSEVSWIPNKHYSGIYGLMKLVLTKTLPANLERVIVLDTDITFATDIAELWAVFHKFKGQQVLGLVENQSDWYLGNLWKNHRPWPALGRGYNTGVILLLLDKLRKMKWEQMWRLTAERELMGMLSTSLADQDIFNAVIKQNPFLVYQLPCFWNVQLSDHTRSEQCYRDVSDLKVIHWNSPKKLRVKNKHVEFFRNLYLTFLEYDGNLLRRELFGCPSETDVNNENLQKQLSELDEDDLCYEFRRERFTVHRTHLYFLHYEFEPSADNTDVTLVAQLSMDRLQMLEAICKHWEGPISLALYLSDAEAQQFLRYAQGSEVLMSRQNVGYHIVYKEGQFYPVNLLRNVAMKHISTPYMFLSDIDFLPMYGLYEYLRKSVIQLDLANTKKAMIVPAFETLRYRLSFPKSKAELLSMLDMGTLFTFRYHVWTKGHAPTNFAKWRTATTPYQVEWEADFEPYVVVRRDCPEYDRRFVGFGWNKVAHIMELDAQEYEFTVLPNAYMIHMPHAPSFDITKFRSNKQYRICLKTLKEEFQQDMSRRYGFAALKYLTAENNS