Key result
Transgenic mice with a downregulated Prkar1a gene developed thyroid follicular hyperplasia, adrenocortical hyperplasia, and other features replicating findings in Carney complex patients.
Population
Transgenic mouse carrying an antisense transgene for Prkar1a exon 2 under the control of a tetracycline…
Design
Preclinical
Authors
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Animal model supports RIalpha as tumor suppressor candidate; leaves open human therapeutic translation pending further studies.
The tTA/X2AS mouse model with downregulated Prkar1a replicates findings in Carney complex patients, supporting RIalpha as a candidate tumor suppressor gene.
Kurt Griffin (2004) studied Carney complex and other PRKAR1A induced lesions. Antisense transgene for Prkar1a exon 2 (X2AS) was evaluated on Development of endocrine and other tumours. Transgenic mice with a downregulated Prkar1a gene developed thyroid follicular hyperplasia, adrenocortical hyperplasia, and other features replicating findings in Carney complex patients.
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