Table 2.

Ikbkap^Δ20/flox and Ikbkap^flox/flox mouse models reproduce features of FD

	FD patients	IkbkapΔ20/flox	Ikbkapflox/flox
Reduced expression of full-length IKAP protein	5–20% of controls in CNS	5% of controls in CNS tissues	10% of controls in CNS tissues
Intrauterine growth retardation	+	+	+
Low birth weight	80% of controls	70% of controls	85% of controls
Poor suck, uncoordinated swallow at birth	+	+	+
Poor weight gain	+	+	+
Short stature	+	+	+
Reduced life-span	+	+	+
Dysautonomic crisis	+	N/A	N/A
Seizure susceptibility	+	+	+
Gastrointestinal dysfunction	+	+	+
Absence of overflow emotional tears	+	N/A	N/A
Optic neuropathy	+	+	+
Tongue: Reduced numbers of fungiform papillae	Smooth tongue	45% reduction	30% reduction
Decreased deep tendon reflexes	+	N/D	N/D
Muscle spindle abnormalities	Impaired function	N/D	Reduced sensory innervation
Absent axon flare following intradermal histamine injection	+	N/D	N/D
Poor coordination/balance	+	+	+
Spinal abnormalities	+	+	+
Hydronephrosis	+	+	+
Decreased temperature perception	+	+	+
Decreased volume of DRGs	+	45% of controls at birth	70% of controls at birth
Decreased neuronal numbers in DRGs in adults	10–20% of controls	75% reduction in nociceptive neurons	20% reduction in nociceptive neurons
Decreased volume of sympathetic ganglia at birth	N/D	45% of controls at birth	70% of controls at birth
Decreased volume of SCGs in adults	30% of controls	N/D	30% of controls
Decreased neuronal numbers in SCGs in adults	10% of controls	N/D	20% of controls

+, present; N/A, not applicable; N/D, not determined.