| First Author | Taghibiglou C | Year | 2011 |
| Journal | Biochem Biophys Res Commun | Volume | 413 |
| Issue | 2 | Pages | 159-63 |
| PubMed ID | 21871872 | Mgi Jnum | J:177533 |
| Mgi Id | MGI:5295350 | Doi | 10.1016/j.bbrc.2011.08.011 |
| Citation | Taghibiglou C, et al. (2011) Sterol regulatory element binding protein-1 (SREBP1) activation in motor neurons in excitotoxicity and amyotrophic lateral sclerosis (ALS): Indip, a potential therapeutic peptide. Biochem Biophys Res Commun 413(2):159-63 |
| abstractText | Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease of motor neurons in which glutamatergic excitotoxicity may participate. A recently characterized downstream effector of glutamatergic excitotoxicity is the activation of the lipid transcription factor sterol regulatory element binding protein-1 (SREBP1). Here we report that in spinal cord tissues of transgenic mouse model, G93A, as well as post-mortem spinal cord specimens of human familial and sporadic ALS, significant activation of SREBP1 following drastic degradation of ER membrane resident protein Insig-1. A TAT-fused short peptide (Indip) to prevent Insig-1 degradation and subsequent SREBP1 activation significantly protected cultured spinal cord neurons against glutamate-induced excitotoxicity. Indip or other SREBP1-pathway modulating compounds may prove beneficial in ALS. |
