Characterization of early markers of disease in the mouse model of mucopolysaccharidosis IIIB

Table 1 Order of and age at behavioral testing per cohort with related patient phenotype and specific assessment in mice

Cohort	Related Patient Phenotype	Specific Assessment	Behavioral Test	Age
1	Developmental delay	Developmental growth assessment	Physical milestone inspection, reflex achievement, weight collection	P6-14
1	Speech delay	Developmental vocalization levels	Maternal isolation-induced USV recordings	P6-10
2	Motor impairment	Developmental gait trajectories	DigiGait assay	P21-30, P60
3	Altered body size trajectory	Bi-weekly weight measurements	–	–
	Health decline	Physical features and posture	Physical exam	P35
	Autistic-like social abnormalities	Sociability and social novelty	Social approach assay	P40
	Vision decline	Visual acuity and contrast	Virtual optomotor system assessment	P47
	Sensory deficits	Sensorimotor gating	Acoustic startle response/PPI task	P53
	Autistic-like social abnormalities	Social hierarchy behaviors	Social dominance tube test	P62
	Reduction in fear	Associative anxiety and conditioning	Fear conditioning test	P67
	Aggression	Aggressive and agonistic behaviors	Resident intruder assay	P96

ISSN: 1866-1955