Table 1.

Mouse models of KD/SBMA

Original reference	Construct promoter- protein (#Q)	Phenotype	Onset	Androgen dependent
Bingham et al. (1995)	Mx-AR(45Q) NSE-AR(45Q)	Very low transgene expression No phenotype	N/A	N/A
La Spada et al. (1998)	AR-AR(45Q)	No phenotype reported, repeat instability	N/A	N/A
Abel et al. (2001) Chevalier-Larsen et al. (2004)	PrP-Δ AR(112Q)	CNS but not muscle inclusions, motor deficits, no obvious neuropathy or myopathy, some male infertility	10–12 weeks	Y
Abel et al. (2001)	NFL-Δ AR(112Q)	Some CNS inclusions, motor deficits, no obvious neuropathy	34 weeks	?
Adachi et al. (2001)	AR-239Q	CNS inclusions, motor deficits, no neuropathy loss	10–12 weeks	N
Katsuno et al. (2002)	CMV/β–actin-AR(97Q)	CNS, muscle inclusions, motor deficits motor deficits, neuropathy; Neurogenic and myogenic myopathy	10–12 weeks	Y
McManamny et al. (2002)	CMV-AR(65Q) CMV-AR(120Q)	Mild atrophy, not fully characterized No inclusions, motor deficits, neuropathy, testis atrophy, myopathy	3 weeks	N
Sopher et al. (2004)	AR-AR(100Q)	CNS inclusions (but not motoneurons), neuropathy, myopathy, reduced VEGF	32–48 weeks	?
Yu et al. (2006a)	AR(113Q) KI	CNS and muscle inclusions, motor deficits neuropathy, testicular atrophy, myopathy transcriptional dysregulation, electrophysiological disturbance	Adult	Y
Monks et al. (2007)	HAS-AR(22Q)	No inclusions, motor deficits, axonopathy myopathy, transcriptional dysregulation, VEGF	Perinatal/10–12 weeks	Y

ΔAR = Truncated AR, CMV = cytomegalovirus promoter, HSA = human skeletal actin promoter (muscle specific), KI = knock-in of polyQ tract, Mx = interferon-induced cellular resistance protein, NFL = neurofilament light chain (neuron specific), NSE = Neuron specific enolase, PrP = prion protein promoter.