Animal and Cellular Models of Barth Syndrome

At present, there are several models of Barth syndrome, each of which has its own unique characteristics. All models show a cardiolipin dysfunction. If you use one of these models, please keep in contact with BSF Science Director, Matthew Toth, so that new, unpublished information can be relayed to you. Please contact the senior authors of the publications for more information.

*Publications that acknowledge financial support contributed by BSF and/or BSF Affiliates.

▼Publications that acknowledge biological samples (and/or information) from Barth families, the Barth Syndrome Registry and Repository (BRR), and/or BSF Affiliates.


1) MOUSE MODELS

The model most frequently mentioned is the knockdown mouse model which was made using a siRNA transgene at the ROSA26 locus to lower the expression of the endogenous mouse tafazzin gene. This mouse model is inducible using doxycycline, and it was made using the proprietary technology of TaconicArtemis, GmbH (Köln, Germany) under contract from BSF. The knockdown mouse model (KD mouse) is available without impediment from Jackson Laboratories (B6.Cg-Gt(ROSA)26Sortm37(H1/tetO-RNAi:Taz)Arte/ZkhuJ (https://www.jax.org/strain/014648). There is also a knockout mouse model of Barth syndrome available from the Strathdee lab (Cadalbert et al., 2015). A list of primary publications using mouse models is below.

2) CELLULAR MODELS

Cellular models of Barth syndrome have used shRNAs in rodent cardiac cells to lower tafazzin gene expression in different primary cell lines. The publications for these models are listed below.

3) DROSOPHILA MODELS

Drosophila models of Barth syndrome utilize the imprecise excision of a P element from stock y. Below is a list of publications.

4) YEAST MODELS

A yeast model of Barth syndrome has also been used extensively. Below is a list of publications.

5) INDUCED PLURIPOTENT STEM CELL MODELS AND MOUSE EMBRYONIC FIBROBLAST MODEL

Human iPS cells and mouse embryonic fibroblasts are also represented in Barth syndrome as shown in the publications below.

6) ZEBRAFISH MODEL

A zebra fish model has also been described.

 

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