B4GALT7 (beta-1,4-galactosyltransferase 7)

2008-05-01  

Identity

HGNC
LOCATION
5q35.3
LOCUSID
ALIAS
EDSP1,EDSSLA,EDSSPD1,XGALT1,XGPT,XGPT1
FUSION GENES

Other Information

Locus ID:

NCBI: 11285
MIM: 604327
HGNC: 930
Ensembl: ENSG00000027847

Variants:

dbSNP: 11285
ClinVar: 11285
TCGA: ENSG00000027847
COSMIC: B4GALT7

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000027847ENST00000029410Q9UBV7
ENSG00000027847ENST00000505433D6RJI5
ENSG00000027847ENST00000505468D6RDJ8
ENSG00000027847ENST00000507061H0Y9D6
ENSG00000027847ENST00000510761D6RA33

Expression (GTEx)

0
5
10
15
20
25
30
35
40
45
50

Pathways

PathwaySourceExternal ID
Glycosaminoglycan biosynthesis - chondroitin sulfate / dermatan sulfateKEGGko00532
Glycosaminoglycan biosynthesis - heparan sulfate / heparinKEGGko00534
Glycosaminoglycan biosynthesis - chondroitin sulfate / dermatan sulfateKEGGhsa00532
Glycosaminoglycan biosynthesis - heparan sulfate / heparinKEGGhsa00534
Metabolic pathwaysKEGGhsa01100
Glycosaminoglycan biosynthesis, linkage tetrasaccharideKEGGhsa_M00057
Glycosaminoglycan biosynthesis, linkage tetrasaccharideKEGGM00057
MetabolismREACTOMER-HSA-1430728
Metabolism of carbohydratesREACTOMER-HSA-71387
Glycosaminoglycan metabolismREACTOMER-HSA-1630316
Heparan sulfate/heparin (HS-GAG) metabolismREACTOMER-HSA-1638091
A tetrasaccharide linker sequence is required for GAG synthesisREACTOMER-HSA-1971475
Chondroitin sulfate/dermatan sulfate metabolismREACTOMER-HSA-1793185

Protein levels (Protein atlas)

Not detected
Low
Medium
High

References

Pubmed IDYearTitleCitations
380256832023Targeting B4GALT7 suppresses the proliferation, migration and invasion of hepatocellular carcinoma through the Cdc2/CyclinB1 and miR-338-3p/MMP2 pathway.0
380256832023Targeting B4GALT7 suppresses the proliferation, migration and invasion of hepatocellular carcinoma through the Cdc2/CyclinB1 and miR-338-3p/MMP2 pathway.0
312783922019Expansion of B4GALT7 linkeropathy phenotype to include perinatal lethal skeletal dysplasia.7
316148622019Severe Peripheral Joint Laxity is a Distinctive Clinical Feature of Spondylodysplastic-Ehlers-Danlos Syndrome (EDS)-B4GALT7 and Spondylodysplastic-EDS-B3GALT6.7
312783922019Expansion of B4GALT7 linkeropathy phenotype to include perinatal lethal skeletal dysplasia.7
316148622019Severe Peripheral Joint Laxity is a Distinctive Clinical Feature of Spondylodysplastic-Ehlers-Danlos Syndrome (EDS)-B4GALT7 and Spondylodysplastic-EDS-B3GALT6.7
269401502016Further defining the phenotypic spectrum of B4GALT7 mutations.16
269401502016Further defining the phenotypic spectrum of B4GALT7 mutations.16
247559492015Expanding the clinical spectrum of B4GALT7 deficiency: homozygous p.R270C mutation with founder effect causes Larsen of Reunion Island syndrome.31
255683252015Probing the acceptor active site organization of the human recombinant β1,4-galactosyltransferase 7 and design of xyloside-based inhibitors.9
247559492015Expanding the clinical spectrum of B4GALT7 deficiency: homozygous p.R270C mutation with founder effect causes Larsen of Reunion Island syndrome.31
255683252015Probing the acceptor active site organization of the human recombinant β1,4-galactosyltransferase 7 and design of xyloside-based inhibitors.9
240522592013Crystal structures of β-1,4-galactosyltransferase 7 enzyme reveal conformational changes and substrate binding.21
240522592013Crystal structures of β-1,4-galactosyltransferase 7 enzyme reveal conformational changes and substrate binding.21
206916852010Molecular characterization of β1,4-galactosyltransferase 7 genetic mutations linked to the progeroid form of Ehlers-Danlos syndrome (EDS).8

Citation

Dessen P

B4GALT7 (beta-1,4-galactosyltransferase 7)

Atlas Genet Cytogenet Oncol Haematol. 2008-05-01

Online version: http://atlasgeneticsoncology.org/gene/50103/b4galt7-(beta-1-4-galactosyltransferase-7)