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2.4.1.B80: alpha-dystroglycan glucuronosyltransferase

This is an abbreviated version!
For detailed information about alpha-dystroglycan glucuronosyltransferase, go to the full flat file.

Word Map on EC 2.4.1.B80

Reaction

UDP-alpha-D-glucuronate
+
alpha-D-xylosyl-(1->3)-[3)-beta-D-glucuronosyl-(1->3)-alpha-D-xylosyl-(1->]n-[O-mannosyl-chain of alpha-dystroglycan]
=
UDP
 +
beta-D-glucuronosyl-(1->3)-alpha-D-xylosyl-(1->3)-[3)-beta-D-glucuronosyl-(1->3)-alpha-D-xylosyl-(1->]n-[O-mannosyl-chain of alpha-dystroglycan]

Synonyms

LARGE1, like-acetylglucosaminyltransferase

ECTree

     2 Transferases
         2.4 Glycosyltransferases
             2.4.1 Hexosyltransferases
                2.4.1.B80 alpha-dystroglycan glucuronosyltransferase

Disease

topprint

Disease on EC 2.4.1.B80 - alpha-dystroglycan glucuronosyltransferase

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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
Arenaviridae Infections
Like-acetylglucosaminyltransferase (LARGE)-dependent modification of dystroglycan at Thr-317/319 is required for laminin binding and arenavirus infection.
Diabetes Mellitus, Type 2
Identification of novel high-impact recessively inherited type 2 diabetes risk variants in the Greenlandic population.
Liver Neoplasms
Clinical significance of LARGE1 in progression of liver cancer and the underlying mechanism.
Lung Neoplasms
High LARGE1 Expression May Predict Benefit from Adjuvant Chemotherapy in Resected Non-Small-Cell Lung Cancer.
Loss of alpha-dystroglycan laminin binding in epithelium-derived cancers is caused by silencing of LARGE.
Meningioma
The human LARGE gene from 22q12.3-q13.1 is a new, distinct member of the glycosyltransferase gene family.
Muscular Dystrophies
Identification and Modeling of a GT-A Fold in the ?-Dystroglycan Glycosylating Enzyme LARGE1.
O-mannosyl phosphorylation of alpha-dystroglycan is required for laminin binding.
POMK regulates dystroglycan function via LARGE1-mediated elongation of matriglycan.
Neoplasms
Clinical significance of LARGE1 in progression of liver cancer and the underlying mechanism.
High LARGE1 Expression May Predict Benefit from Adjuvant Chemotherapy in Resected Non-Small-Cell Lung Cancer.
The human LARGE gene from 22q12.3-q13.1 is a new, distinct member of the glycosyltransferase gene family.
Rhabdomyosarcoma
Exogenous expression of the glycosyltransferase LARGE1 restores ?-dystroglycan matriglycan and laminin binding in rhabdomyosarcoma.
Walker-Warburg Syndrome
O-mannosyl phosphorylation of alpha-dystroglycan is required for laminin binding.