Table 2.

Clinical and biochemical features of human HD-AIP and R167Q^+/+ mice

Human HD-AIP features	R167Q+/+ mice
Early onset of neurological impairment	+
Tremor	+
Ataxia	+
Dystonic posture	+
Early demisea	+
Short stature	+
Constitutively elevated urinary ALA and PBG	+
Porphyrinuria	+
Decreased CNS myelination	+

+, Present in the R167Q^+/+ mice.

^aApproximately 35% of R167Q^+/+ mice die before P30, but those that survive have a normal life span.