Abcb4-KO(FVB)

Nomenclature

FVB-Abcb4em1Smoc

Cat. NO.

NM-KO-200584

Strain State

Embryo cryopreservation

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Gene Summary

Gene Symbol
Abcb4

Synonyms

Mdr2; Pgy2; Pgy-2; mdr-2

NCBI ID

18670

MGI ID

97569

Ensembl ID

ENSMUSG00000042476

Pubmed

Abcb4

Human Ortholog

ABCB4

Model Description

Exon 3-4 of Abcb4 gene was deleted to generate Abcb4 knockout mice.

Research Application：Diseases associated with ABCB4 include Cholestasis, Progressive Familial Intrahepatic, 3 and Gallbladder Disease 1. 

Disease Connection

Cholecystitis

Phenotype(s)

MGI:3840644

Reference(s)

Lammert F, Wang DQ, Hillebrandt S, Geier A, Fickert P, Trauner M, Matern S, Paigen B, Carey MC, Spontaneous cholecysto- and hepatolithiasis in Mdr2-/- mice: a model for low phospholipid-associated cholelithiasis. Hepatology. 2004 Jan;39(1):117-28

Cholestasis

Phenotype(s)

MGI:5526018

Reference(s)

Hochrath K, Ehnert S, Ackert-Bicknell CL, Lau Y, Schmid A, Krawczyk M, Hengstler JG, Dunn J, Hiththetiya K, Rathkolb B, Micklich K, Hans W, Fuchs H, Gailus-Durner V, Wolf E, de Angelis MH, Dooley S, Paigen B, Wildemann B, Lammert F, Nussler AK, Modeling hepatic osteodystrophy in Abcb4 deficient mice. Bone. 2013 Aug;55(2):501-11

Hepatocellular Carcinoma

Phenotype(s)

MGI:2653844

Reference(s)

Mauad TH, van Nieuwkerk CM, Dingemans KP, Smit JJ, Schinkel AH, Notenboom RG, van den Bergh Weerman MA, Verkruisen RP, Groen AK, Oude Elferink RP, van der Valk MA, Borst P, Offerhaus GJ, Mice with homozygous disruption of the mdr2 P-glycoprotein gene. A novel animal model for studies of nonsuppurative inflammatory cholangitis and hepatocarcinogenesis. Am J Pathol. 1994 Nov;145(5):1237-45

Intrahepatic Cholestasis

Phenotype(s)

MGI:3694478

Reference(s)

Li Z, Agellon LB, Vance DE, The role of phosphatidylethanolamine methyltransferase in a mouse model of intrahepatic cholestasis. Biochim Biophys Acta. 2011 Apr;1811(4):278-83

Primary Sclerosing Cholangitis

Phenotype(s)

MGI:5618431

Reference(s)

Strack I, Schulte S, Varnholt H, Schievenbusch S, Tox U, Wendland K, Steffen HM, Drebber U, Dienes HP, Odenthal M, beta-Adrenoceptor blockade in sclerosing cholangitis of Mdr2 knockout mice: antifibrotic effects in a model of nonsinusoidal fibrosis. Lab Invest. 2011 Feb;91(2):252-61

Validation Data

image.png

Fig1. Serum biochemistry of Abcb4-KO mice (n=3 female and 3 male).

image.png

Fig2. Significant bile duct hyperplasia and interstitial focal cell infiltration were observed in the liver of Abcb4-KO mice.

(A) Liver were collected from FVB wild-type (+/+) and Abcb4-KO mice, and analyzed by HE staining. (B) Pathological analysis of the liver in Abcb4-KO mice.

image.png

Fig3. Collagen fiber hyperplasia were observed in the liver of Abcb4-KO mice.

(A) Liver were collected from FVB wild-type (+/+) and Abcb4-KO mice, and analyzed by Masson staining. (B) Quantification of the Collagen volume fraction (CVF) .

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