Table 1.

Histopathological and molecular findings in Speg- and Mtm1-mutant mouse models

Findings	Speg-KO (Spegfl/fl: MCK-cre+)	Mtm1-KO (Mtm1−/y)
Triad structure and function	Poor integrity, low number of triads and calcium mishandling (33)	Fewer triads and abnormal longitudinally oriented T-tubules, impaired SR calcium release (27)
Triadic protein abnormalities	Reduced RyR1 and triadin protein, mislocalization of DHPRα1, SERCA1 and triadin (this study)	A 3-fold reduction of RyR1 protein, ~30% decrease of DHPRα1 and 6-fold increase of DHPRβ1 in microsomal preparations (27); disorganization of DHPR and RyR1 labeling (44)
Desmin aggregation	Cytoplasmic desmin aggregates and increased insolubility (this study)	Increased protein level and insolubility of desmin, desmin aggregation (31)
Focal adhesion defects	Cytoplasmic accumulation of vinculin and β1 integrin, accumulation of β1 integrin at early endosomes (this study)	Increased protein levels of laminin, vinculin and β1 integrin; internalized vinculin and β1 integrin; and accumulation of β1 integrin at early endosomes (41)
Others	Fewer satellite cells and delayed muscle regeneration in response to injury (58)	Abnormalities in the number and behavior of myogenic cells with decreased proliferation and increased apoptosis (30)