Sarcospan: a small protein with large potential for Duchenne muscular dystrophy

Skeletal Muscle

Table 1 Sarcospan- and α7 integrin-double nulls display severe growth and muscle phenotypes

Genotype	Survival Analysis^a	Kyphosis^a	Myofiber CSA^a	Central Nucleation^a	Utrophin^a	Dystrophin^a	Integrin^a	Laminin-Binding^a	Signaling^a	Specific Force^a
Wild type	100%	No	Normal	Normal	100%	100%	100%	100%	P-Akt/Akt: 100%	Normal (EDL, Soleus, Diaphragm)
Wild type	100%	No	Normal	Normal	100%	100%	100%	100%	P-IGFR/IGFR: 100%	Normal (EDL, Soleus, Diaphragm)
SSPN null	100%	No	Normal	Normal	31%	47%	293%	69%	P-Akt/Akt: 49%	Normal (EDL, Soleus); Decrease (Diaphragm)
SSPN null	100%	No	Normal	Normal	31%	47%	293%	69%	P-IGFR/IGFR: 80%	Normal (EDL, Soleus); Decrease (Diaphragm)
Itga7 null	95%	Minor	Normal	Normal	102%	48%	Absent	85%	P-Akt/Akt: 95%	Decrease (Diaphragm)
Itga7 null	95%	Minor	Normal	Normal	102%	48%	Absent	85%	P-IGFR/IGFR: 99%	Decrease (Diaphragm)
DKO	60%	Severe	Decrease	Increase	44%	34%	Absent	28%	P-Akt/Akt: 40%	Severe Decrease (Diaphragm)
DKO	60%	Severe	Decrease	Increase	44%	34%	Absent	28%	P-IGFR/IGFR: 35%	Severe Decrease (Diaphragm)

^aMeasurements represented relative to wild type. DKO, double knockout; Itga7, α7 integrin.

ISSN: 2044-5040