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. Author manuscript; available in PMC: 2010 Dec 1.
Published in final edited form as: J Investig Med. 2009 Dec;57(8):837–841. doi: 10.231/JIM.0b013e3181af59d6

Table 1. Comparison of FXTAS with the CGG KI Mouse.

  • CGG Expansions

    • Human FXTAS – 55-200 CGG repeats, repeat instability3

    • CGG KI Mouse – 70 to >300 CGG repeats, modest repeat instability6

  • Elevated FMR1 mRNA

    • Human FXTAS – 2-8 fold increase7-9

    • CGG KI Mouse – 3-5 fold increase5

  • FMRP

    • Human FXTAS – reduced in several brain regions10

    • CGG KI Mouse – normal to slightly reduced with long repeats11

  • Motor Impairment

    • Human FXTAS – tremor/ataxia, postural sway, Parkinsonism12

    • CGG KI Mouse – impaired on rotarod test of motor coordination13

  • Cognitive Impairment

    • Human FXTAS – poor memory, anxiety, depression, social phobia7,14

    • CGG KI Mouse – spatial memory deficits, open field anxiety13

  • Intranuclear Ubituitin-positive Inclusions

    • Human FXTAS – in neurons and astrocytes, correlated with CGG length15

    • CGG KI Mouse – primarily in neurons, few in astrocytes5