TABLE 3.

Correlation between MS‐related animal models and clinical/pathological features of progressive MS

MS‐related animal model	Hypothesized driving force for disease progression	Clinical/pathological features of progressive MS
		Presence of ELFs	Grey matter demyelination and/or brain atrophy	Diffuse white matter damage	Profound oxidative injury	Progressive worsening of motor/cognitive functions	Moderate parenchymal immune cell infiltration	Moderate BBB integrity loss
MOG‐EAE	Inflammation			Moderate				Severe
PLP‐EAE	Inflammation			Moderate				Severe
MP4‐EAE	Inflammation			Moderate				Severe
ABH‐EAE	Inflammation							Severe
2D2×Th	Inflammation			Moderate				Severe
Cup/EAE	Neurodegeneration							Unknown
Cup	Neurodegeneration

Characteristics can be studied (green) or cannot be studied (red).

MOG‐EAE: C57BL/6 mice immunized with MOG_35‐55 peptides develop a monophasic, chronic disease course.

PLP‐EAE: SJL mice immunized with PLP_139–151 peptides develop a relapsing–remitting disease course.

MP4‐EAE: C57BL/6 mice immunized with an MPB‐PLP fusion protein (MP4) induces a B‐cell‐dependent pathology.

ABH‐EAE: Biozzi ABH mice immunized with spinal cord homogenate develop a disease course with relapsing–remitting episodes and a secondary progressive disability.

2D2×Th: A spontaneous EAE model derived from the crossbreeding of TCR transgenic mice (C57BL/6 2D2 MOG_35–55‐specific) and MOG‐specific Ig heavy‐chain knock‐in mice (referred to as Th mice).

Cup/EAE: MOG‐EAE mice predisposed to a 3‐week cuprizone intoxication and 2‐week normal chow develop inflammatory demyelination in the forebrain.

Cup: C57BL/6 mice intoxicated with cuprizone, a reagent inducing apoptosis in mature oligodendrocytes, developing innate immune activation within the CNS followed by demyelination.