Basic Information

Strain name
Common name
B-hB4GALT1 mice
Catalog number
B4GAL-T1, CDG2D, CLDLFIB, GGTB2, GT1, GTB, beta4Gal-T1
NCBI gene ID


  • B4GALT1 is a member of the β-1, 4-galactosyltransferase family, which has membrane and secretory isoforms. B4GALT1 is a widely studied glycosyltransferase, a type II membrane-bound glycoprotein that transfers galactose from uridine diphosphate galactose (UDP-Gal) to a specific glycoprotein substrate. B4GALT1 is distributed in the Golgi apparatus, some cell surfaces, and body fluids in vivo. B4GALT1 in the Golgi apparatus catalyzes the formation of β-1, 4-glucoside bonds between galactose and N-acetylglucosamine. B4GALT1 on the cell surface is a cell adhesion molecule involved in cell-matrix and cell-cell interactions. The deficiency of B4GALT1 is the cause of 2D congenital disorders of glycosylation. In IgA nephropathy, the B4GALT1 expression in glomerular is increased.
  • Protein expression analysis: B4GALT1 was detectable in liver, kidney and colon of both homozygous B-hB4GALT1 mice and wild-type mice, as anti-B4GALT1 antibody was cross-reactive between human and mouse.
  • mRNA expression analysis: Mouse B4galt1 was only detectable in kidney of wild-type C57BL/6 mice. Human B4GALT1 was detectable in kidney of homozygous B-hB4GALT1 mice.

Targeting strategy

Gene targeting strategy for B-hB4GALT1 mice. The exons 1-6 of mouse B4galt1 gene that encode extracellular domain main and 3’ UTR region are replaced by human counterparts in B-hB4GALT1 mice. The genomic region of mouse B4galt1 gene that encodes cytoplasmic portion and transmembrane domain is retained. The promoter and 5’UTR region of the mouse gene are also retained. The chimeric B4GALT1 expression is driven by endogenous mouse B4galt1 promoter, while mouse B4galt1 gene transcription and translation will be disrupted.

mRNA expression analysis

Strain specific analysis of B4GALT1 mRNA expression in wild-type C57BL/6 mice and B-hB4GALT1 mice by RT-PCR. Kidney RNA were isolated from wild-type C57BL/6 mice (+/+) and homozygous B-hB4GALT1 mice (H/H), then cDNA libraries were synthesized by reverse transcription, followed by PCR with mouse or human B4GALT1 primers. Mouse B4galt1 mRNA was detectable in wild-type mice. Human B4GALT1 mRNA was detectable only in homozygous B-hB4GALT1 mice but not in wild-type mice.

Protein expression analysis

Western blot analysis of B4GALT1 protein expression in homozygous B-hB4GALT1 mice. Various tissue lysates were collected from wild-type C57BL/6 mice (+/+) and homozygous B-hB4GALT1 mice (H/H), and then analyzed by western blot with cross reactive anti-B4GALT1 antibody (Abcam ab121326). 40 μg total proteins were loaded for western blotting analysis. B4GALT1 was detected in liver, kidney and colon of both homozygous B-hB4GALT1 mice and wild-type mice.

Inhibitory efficiency of the HSD17B13 targeted nucleic acid drugs

The inhibitory efficiency of the nucleic acid drugs against human HSD17B13 mRNA in liver tissue in homozygous B-hHSD17B13 mice. B-hHSD17B13 mice were randomly divided into two groups (8 weeks old). The human HSD17B13 targeted nucleic acid drugs (synthesized according to patents) and PBS were administered to the mice individually. The nucleic acid drugs were administered in the form of PBS aqueous solution. The mice were sacrificed on day 7, and the liver tissue was collected to detect the expression level of human HSD17B13 mRNA by qPCR. (A) The schematic diagram of experimental processing. (B) The expression of human HSD17B13 mRNA in liver after treatment. The inhibition rate in the treatment group was 75%. The human HSD17B13 in the treatment group (G2) was reduced compared to the control group (G1), demonstrating that B-hHSD17B13 mice provide a powerful preclinical model for in vivo evaluation of human HSD17B13 targeted nucleic acid drugs. Values are expressed as mean ± SEM.

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