Abcb4-KO(FVB)

系統名

FVB-Abcb4em1Smoc

SMOC番号

NM-KO-200584

維持形態

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遺伝子の概要

Gene Symbol
Abcb4

Synonyms

Mdr2; Pgy2; Pgy-2; mdr-2

NCBI ID

18670

MGI ID

97569

Ensembl ID

ENSMUSG00000042476

Pubmed

Abcb4

Human Ortholog

ABCB4

説明

Exon 3-4 of Abcb4 gene was deleted to generate Abcb4 knockout mice.
応用分野：Diseases associated with ABCB4 include Cholestasis, Progressive Familial Intrahepatic, 3 and Gallbladder Disease 1. 

病気の予測

Cholecystitis

表現型

MGI:3840644

参考文献

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

表現型

MGI:5526018

参考文献

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

表現型

MGI:2653844

参考文献

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

表現型

MGI:3694478

参考文献

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

表現型

MGI:5618431

参考文献

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

表現型データ

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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