Neuropathology of RAN translation proteins in fragile X-associated tremor/ataxia syndrome

Table 1 Summary of antibody staining in control and FXTAS patient tissue

Brain Region	Case		Age	Sex	UB	p62	UBQLN2	Nterminal FMRpolyG	Cterminal FMRpolyG
Hippocampus	Control	1	59	N/A	++	+	++	+	+
		2	71	M	+	–	+	–	–
		3	39	M	–	+	–	–	–
		4	47	M	–	+	+	–	–
	FXTAS	1	74	M	++++	++++	+++	++++	+
		2	78	M	+++	++	++++	++++	+
		3	80	M	++++	++	+	++	++
		4	69	M	++++	+++	++	++	++
Cortex	Control	2	71	M	+	–	–	–	–
		4	47	M	+	+	+	+	–
		5	84	M	++++	–	–	+++	–
		6	78	M	++++	–	–	++++	–
	FXTAS	1	74	M	++++	++	+	++++	+
		2	78	M	++	++	–	++++	+
		3	80	M	++++	+	+	+	–

-, negative; +, low-intensity staining; ++, moderate-intensity staining; +++, high-intensity staining; ++++, high-intensity and frequent staining. UBQLN2 ubiquilin 2, N/A not available; M, male

ISSN: 2051-5960