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. Author manuscript; available in PMC: 2013 Mar 1.
Published in final edited form as: J Neurosci Res. 2011 Nov 8;90(3):706–714. doi: 10.1002/jnr.22786

Figure 1.

Figure 1

Establishment of transgenic mouse model and instability of the pentanucleotide repeats.

A. The cytomegalovirus (CMV) promoter sequences in the plasmid pcDNA3.1-hygro-lacZ (Invitrogen) were replaced with the MfeI /BamHI fragment of the rat prion promoter sequences. 500 ATTCT repeats were inserted in the 3′ UTR of LacZ.

B. Presence and integrity of 500 ATTCT repeats as analyzed by Southern blot (arrowhead). The smear phenomenon in the patient lane is due to somatic mosaicism of the expanded allele. Three founders and their F1 animals had the expanded allele.

C. Repeat size analysis of repeat primed PCR (RP-PCR) products from the founders and their progeny by acrylamide gel electrophoresis showed variability in the ATTCT repeat expansion size. Lane 1 – positive control from plasmid DNA, Lane 2 - wildtype mouse, Lane 3 – Founder 1, Lane 4 – Founder 2, Lane 5 – F1 from founder 1, Lane 6 – F1 from Founder 2, Lane 7 – F2 from Founder 2, Lane 8-F1 from founder 3, Lane 9-14 F2 from founder 3.

D. Real-time RT-PCR analysis revealed that the transgene is widely expressed in the central nervous system (CNS) with the highest level in the hippocampus. Expression was also seen in non-CNS derived tissues with the highest levels present in the heart.