Sarcospan (Kras Oncogene-Associated Gene) Proteins (SSPN)

Human Sarcospan (Kras Oncogene-Associated Gene) (SSPN) interaction partners

1. Our data suggest TWIST1 and SSPN to be the functionally relevant androgenetic alopecia genes at the 7p21.1 and 12p12.1 risk loci, respectively.

2. When human SSPN is expressed at three-fold levels in mdx mice, this increase in adhesion complex abundance improves muscle membrane stability, preventing many of the histopathological changes associated with Duchenne muscular dystrophy

3. Analysis of sarcospan as a candidate gene for congenital fibrosis of the extraocular muscles type 1.

4. Frequent polymorphism of peroxisome proliferator activated receptor gamma gene in colorectal cancer containing wild-type K-ras gene.

5. Sarcospan plays an important mechanical role in stabilizing the dystrophin-glycoprotein complex.

6. crystal structure of Imp beta (127-876) in complex SPN1 (1-65) at 2.8-A resolution reveals that Imp beta adopts an open conformation, which is unique for a functional Imp beta/cargo complex, and resembles the conformation of the Imp beta/RanGTP complex

7. decreased expression of sarcospan at the Fukuyama congenital muscular dystrophy myofiber surfaces.

8. Human SSPN in transgegnic mice directly interacts with utrophin-glycoprotein comlpex and functions to stabilize utrophin protein without increasing utrophin transcription.

Mouse (Murine) Sarcospan (Kras Oncogene-Associated Gene) (SSPN) interaction partners

1. An alternatively spliced isoform of sarcospan localizes to the sarcoplasmic reticulum in skeletal muscle. It is absent in Sgcd-/- skeletal muscle, a murine model for limb girdle muscular dystrophy 2F.

2. these results demonstrate that SSPN overexpression is well tolerated in mdx mice and improves sarcolemma defects that underlie skeletal muscle and pulmonary dysfunction in Duchenne muscular dystrophy

3. regulates cardiac isoproterenol response and prevents Duchenne muscular dystrophy-associated cardiomyopathy

4. Sarcospan-mediated amelioration of muscular dystrophy in mouse model is dependent on the presence of both utrophin and alpha7beta1 integrin.

5. SSPN is a necessary component of dystrophin and Utr function and that SSPN modulation of integrin signaling is required for extracellular matrix attachment and muscle force development.

6. Sarcospan-null mice displayed delayed differentiation after cardiotoxin injury caused by loss of utrophin and Akt signaling

7. Sspn is downregulated in brain during hypoxia. Knockdown of Sspn increased cultured cell death under hypoxia.