Pathophysiology and Treatment of Canavan Disease
Sign Up to like & getrecommendations! Published in 2018 at "Neurochemical Research"
DOI: 10.1007/s11064-018-2693-6
Abstract: Canavan disease is a recessively inherited vacuolar leukodystrophy caused by ASPA mutations [1–3]. ASPA encodes aspartoacylase, an oligodendroglial enzyme required for cleavage of the abundant brain amino acid N-acetyl-l-aspartate (NAA) to acetate and l-aspartate [4].… read more here.
Keywords: treatment canavan; pathophysiology treatment; canavan; canavan disease ... See more keywords
Next generation sequencing of RNA reveals novel targets of resveratrol with possible implications for Canavan disease.
Sign Up to like & getrecommendations! Published in 2019 at "Molecular genetics and metabolism"
DOI: 10.1016/j.ymgme.2018.10.004
Abstract: Resveratrol (RSV) is a small compound first identified as an activator of sirtuin 1 (SIRT1), a key factor in mediating the effects of caloric restriction. Since then, RSV received great attention for its widespread beneficial… read more here.
Keywords: sequencing rna; disease; rsv; canavan disease ... See more keywords
Redirecting N-acetylaspartate metabolism in the central nervous system normalizes myelination and rescues Canavan disease.
Sign Up to like & getrecommendations! Published in 2017 at "JCI insight"
DOI: 10.1172/jci.insight.90807
Abstract: Canavan disease (CD) is a debilitating and lethal leukodystrophy caused by mutations in the aspartoacylase (ASPA) gene and the resulting defect in N-acetylaspartate (NAA) metabolism in the CNS and peripheral tissues. Recombinant adeno-associated virus (rAAV)… read more here.
Keywords: disease; metabolism central; redirecting acetylaspartate; canavan disease ... See more keywords