. ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration. Nat Neurosci. 2008 Apr;11(4):420-2. PubMed.


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  1. This is a thorough and important work, confirming and extending our original findings of dysfunction and structural damage, vascular leakage and edema, in the blood-brain barrier (BBB) and blood-spinal cord barrier (BSCB) even in early-symptomatic G93A ALS mice (Garbuzova-Davis et al., 2007). The authors found BSCB damage in presymptomatic mice and in other ALS mouse models expressing different SOD mutations. Importantly, as we already know that inflammatory processes can alter the BBB, damage was noted in mice prior to the onset of inflammation. This also may have implications for other neurodegenerative diseases such as Alzheimer’s and Parkinson’s, where BBB damage has already been identified in patients. This finding, and our previous findings, may lead to revised pharmaceutical treatments for ALS, some of which assume a functional, intact BBB/BSCB. However, the article leaves open the questions of which disease mechanisms are affecting the BBB/BSCB and, our current focus, how the BBB/BSCB may be repaired.


    . Ultrastructure of blood-brain barrier and blood-spinal cord barrier in SOD1 mice modeling ALS. Brain Res. 2007 Jul 9;1157:126-37. PubMed.