LARGE1
LARGE xylosyl- and glucuronyltransferase 1 | KIAA0609, LARGE

This gene encodes a member of the N-acetylglucosaminyltransferase gene family. It encodes a glycosyltransferase which participates in glycosylation of alpha-dystroglycan, and may carry out the synthesis of glycoprotein and glycosphingolipid sugar chains. It may also be involved in the addition of a repeated disaccharide unit. The protein encoded by this gene is the glycotransferase that adds the final xylose and glucuronic acid to alpha-dystroglycan and thereby allows alpha-dystroglycan to bind ligands including laminin 211 and neurexin. Mutations in this gene cause several forms of congenital muscular dystrophy characterized by cognitive disability and abnormal glycosylation of alpha-dystroglycan. Alternative splicing of this gene results in multiple transcript variants that encode the same protein. [provided by RefSeq, May 2018]

Member of: DE-4 DE-4.18
Biological processes 43 terms
Golgi apparatus (GO:0005794)Golgi apparatus (GO:0005794)Golgi apparatus (GO:0005794)Golgi apparatus (GO:0005794)Golgi membrane (GO:0000139)Golgi membrane (GO:0000139)N-acetylglucosamine metabolic process (GO:0006044)UDP-xylosyltransferase activity (GO:0035252)UDP-xylosyltransferase activity (GO:0035252)acetylglucosaminyltransferase activity (GO:0008375)glucuronosyltransferase activity (GO:0015020)glucuronosyltransferase activity (GO:0015020)glucuronosyltransferase activity (GO:0015020)glucuronosyltransferase activity (GO:0015020)glycoprotein biosynthetic process (GO:0009101)glycoprotein biosynthetic process (GO:0009101)glycosphingolipid biosynthetic process (GO:0006688)glycosyltransferase activity (GO:0016757)glycosyltransferase activity (GO:0016757)hexosyltransferase activity (GO:0016758)hexosyltransferase activity (GO:0016758)manganese ion binding (GO:0030145)manganese ion binding (GO:0030145)muscle cell cellular homeostasis (GO:0046716)muscle cell cellular homeostasis (GO:0046716)negative regulation of muscle cell apoptotic process (GO:0010656)neuromuscular junction (GO:0031594)plasma membrane (GO:0005886)positive regulation of Rac protein signal transduction (GO:0035022)positive regulation of phosphatidylinositol 3-kinase/protein kinase B signal transduction (GO:0051897)positive regulation of skeletal muscle acetylcholine-gated channel clustering (GO:1904395)protein O-linked glycosylation (GO:0006493)protein O-linked glycosylation via mannose (GO:0035269)protein O-linked glycosylation via mannose (GO:0035269)protein O-linked glycosylation via mannose (GO:0035269)protein binding (GO:0005515)skeletal muscle organ development (GO:0060538)skeletal muscle organ development (GO:0060538)skeletal muscle tissue regeneration (GO:0043403)skeletal muscle tissue regeneration (GO:0043403)xylosyltransferase activity (GO:0042285)xylosyltransferase activity (GO:0042285)xylosyltransferase activity (GO:0042285)
Expression (TPM)
LARGE1 — as a Regulated Gene

TFs regulating LARGE1 0 TFs

Transcription factors with Perturb-seq knockdown data for LARGE1. The Binding column indicates whether any binding evidence exists for this TF–gene pair (ChIP-seq or motif footprint peaks). The Mean coef is the average Perturb-seq regression coefficient across active gRNAs (positive = LARGE1 upregulated upon KD; negative = downregulated). The Outlier column indicates whether this gene is in the top or bottom 5% of all TF knockdown effects.

Data: Effect:
TF Mean coef Binding Outlier TF→Gene link

Elements linked to LARGE1

Open chromatin peaks (ATAC-seq) in the genomic neighbourhood of LARGE1, linked by TSS proximity or chromatin conformation (Multiome / HiCAR). Each element overlaps at least one TF ChIP-seq binding site — the TFs column shows how many distinct TFs bind that element.

Accessibility Element Dist. to TSS Link type TFs
chr22:33,850,777–33,851,374 71.7 kb Distal (>10kb) Multiome 87
chr22:33,919,375–33,922,666 1.7 kb Proximal (<10kb) Multiome 656
chr22:33,923,773–33,923,989 932 bp At TSS 108
chr22:33,931,455–33,931,715 8.6 kb Proximal (<10kb) 18
chr22:35,134,690–35,135,283 1212.0 kb Distal (>10kb) Multiome HiCAR 87

Genome Browser

Genomic view of the LARGE1 locus showing ATAC-seq accessibility and RNA-seq expression across the ESC → DE time course, together with TF binding peaks and element-to-TSS loop connections.

chr22:33,840,777 – 35,145,283
Proximal 1 kb Distal 10 kb Multiome HiCAR ATAC-seq RNA-seq