Table 4.

Immunohistochemical findings

		Muscle	Myotilin	α-BC	Desmin	DYS-2	SMI-31	TDP-43	p62	Ubiquitin	LAMP-2	VCP	FHL1	Membr prot	LC3	HSPB8	DNAJB6	Ubiquilin2	Cathepsin B	Filamin C	BAG3	α-actinin	Teletonin	Actin	SERCA	α-tubulin	SMI310	Proteasome	β-amyloid	APP	WIP1
FF1
	III-6	VL			+				–	–																	+		–	–	–
	III-8	S	++	++	+	++		++	++			+
	III-12	VL
		GLU			+	+
		VL	+	+	+	+				–		–		–
	IV-7	GM	++	++	+	++				++
		GM	++	++	++	++	++	++	++	++	–	++	–		++			++	–	–	++	+		–			++	+	–	–	–
	IV-8	GM	++	++	++	+		++	++	++	–	–	–		++			–	–	–	++	+		–		–	++	+	–	–	–
	IV-9	VL	–	+	+	+								–													–	+	–	–	–
		VL	+	+	+	–		++	++			–									++	+	+	–	–	–
	IV-10	VL	++	++	+	+	–		+	+		–		–
		VL	++	++	+	+	–	+	+	++	–	–			+				–	–		–	–	+	+	–	+		–	–	–
FF2
	III-4	VL			–		–
	III-5	VL			–		–			–
	III-7	VL			+	+	+			+
FF3
	IV-1	VL				–				++				–
		GL	++	++	++	+				++				–
FF4
	III-2	VL	++	++	++	++	++	++	++	++	–	++	–		++	+	+	++	+	–	++							+	–	+
	IV-1	VL			+	++				++
FF5
	IV-1	VL	++	++	+	+	++	++	++	–	–	–	–			+	+				+						++		–	–	–
FF6
	IV-1	VL	+	–	+	–	+/–	+/–	+	+	–	+	–		+		+	+/–	–

–, normal, no immunoreactivity; +, present immunoreactivity; ++, prominent immunoreactivity