Abcb4-KO(FVB)

品系全名

FVB-Abcb4em1Smoc

目录号

NM-KO-200584

品系状态

活体

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基因信息

基因名
Abcb4

基因曾用名

Mdr2; Pgy2; Pgy-2; mdr-2

染色体位置

5

NCBI ID

18670

MGI ID

97569

Ensembl ID

ENSMUSG00000042476

Pubmed

Abcb4

人类同源基因

ABCB4

品系描述

敲除Abcb4基因exon 3-4，建立Abcb4基因敲除小鼠模型。
应用领域：胆汁等疾病相关研究

疾病预测

胆囊炎
Cholecystitis

近似模型的表型

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

胆汁淤积
Cholestasis

近似模型的表型

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

肝内胆汁淤积
Intrahepatic Cholestasis

近似模型的表型

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

肝细胞癌
Hepatocellular Carcinoma

近似模型的表型

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

原发性硬化性胆管炎
Primary Sclerosing Cholangitis

近似模型的表型

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

验证数据

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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