Table 1.

Conformation specific antibodies recognize regions of SOD1 exposed upon misfolding.

Antibody	SOD1 epitope (aa)	Reactive for FALS SOD1	Reactive for SALS SOD1		Therapeutic benefits	Reference
			Patient tissue	Modified SOD1
B8H10e	57–78a	+	ndb	nd	nd	Gros-Louis et al., 2010; Pickles et al., 2013
C4F6e	80–118	+	+	+ (ox)	nd	Urushitani et al., 2006; Bosco et al., 2010; Prudencio and Borchelt, 2011; Brotherton et al., 2012; Pickles et al., 2013
MS785	6–16	+	nd	+c	nd	Fujisawa et al., 2012
D3H5	24–55a	+	nd	+ (apo)	+	Gros-Louis et al., 2010
A5C3e	80–118a	+	nd	nd	−	Gros-Louis et al., 2010
SEDI	143–151	+	−	+ (ox)	+d	Rakhit et al., 2007; Liu et al., 2009, 2012; Kerman et al., 2010; Prudencio and Borchelt, 2011; Mulligan et al., 2012
USOD	42–48	+	−	− (ox)	nd	Kerman et al., 2010; Mulligan et al., 2012
DSE2 (3H1)	125–142	+	+	nd	nd	Vande Velde et al., 2008; Grad et al., 2011; Pokrishevsky et al., 2012
4–20Ra-ab	4–20	+	+	nd	nd	Jonsson et al., 2004; Forsberg et al., 2010
57–72Ra-ab	57–72	+	+	nd	nd	Stewart et al., 2006; Forsberg et al., 2010, 2011
131–153Ra-ab	131–153	+	+	nd	nd	Jonsson et al., 2004; Forsberg et al., 2010, 2011

Misfolded SOD1-specific antibodies highlight regions in SOD1 that become exposed as a result of misfolding.

^aDetermined using similar methodology as in C4F6 epitope mapping (Bosco et al., 2010) (unpublished data, Bosco DA).

^bnd, Not determined.

^cSerum starvation in cultured cells results in SOD1 reactivity with antibody.

^dBenefits observed from active immunization with SOD1 peptide recognized by SEDI.

^eCommercially Available.