Results 81 to 90 of about 1,111 (177)
PLoS ONE, 2013 Cancer is associated with globally hypoacetylated chromatin and considerable attention has recently been focused on epigenetic therapies. N-acetyl-L-aspartate (NAA), the primary storage form of acetate in the brain, and aspartoacylase (ASPA), the enzyme ...
Patrick M Long +7 more
doaj +1 more source
Use of localized proton nuclear magnetic resonance spectroscopy in Canavan's disease
The Turkish Journal of Pediatrics, 1998 Canavan's disease is characterized by megalencephaly, leukodystrophy and early motor and mental retardation. On computerized tomography and magnetic resonance imaging, severe changes compatible with white matter disease due to demyelination is ...
N Aydinli, M Calişkan, M Calay, M Ozmen
doaj
, 2004 Subscription resource available via Digital Dissertations only.The doctoral studies described in this thesis involve the phenotypic characterization of the tremor rat, an animal model of Canavan disease, and a proof of principle gene transfer study in ...
McPhee, Scott William John
core
Recovery of Activity and Stability for Clinical Mutants of Aspartoacylase
The FASEB Journal, 2012 Canavan disease is a fatal neurological disorder caused by mutations in the aspA gene, leading to an altered aspartoacylase with diminished catalytic activity. These clinical mutations are found at sites located throughout the enzyme, with majority found far from the catalytically important amino acids. This suggests that destabilization of the protein
Stephen Penalosa Zano +4 more
openaire +1 more source
THE MOLECULAR MECHANISMS AFFECTING N-ACETYLASPARTATE HOMEOSTASIS FOLLOWING EXPERIMENTAL GRADED TRAUMATIC BRAIN INJURY. [PDF]
, 2014 To characterize the molecular mechanisms of N-acetylaspartate (NAA) metabolism following traumatic brain injury (TBI), we measured the NAA, ATP and ADP concentrations and calculated the ATP/ADP ratio at different times from impact, concomitantly ...
Di Pietro, Valentina +20 more
core +1 more source
, 2015 Canavan disease is caused by inactivating ASPA (aspartoacylase) mutations that prevent cleavage of N-acetyl-L-aspartate (NAA), resulting in marked elevations in central nervous system (CNS) NAA and progressively worsening leukodystrophy.
McDonough, Jennifer +19 more
core +1 more source
, 2018 We present the results of molecular modeling of conformational changes in the Y231C and F295S mutants of human aspartoacylase (hAsp), which allow us to propose a mechanism of allosteric regulation of enzyme activity of these protein variants.
Ekaterina D. Kots +5 more
core +1 more source
The function of Aspartoacylase in white and brown adipose tissue
, 2013 Die Aspartoacylase (Aspa) ist dafür verantwortlich N-acetylaspartat (NAA), das zuvor im Mitochondrium mit Hilfe der N-acetyl-transferase-8-like (Nat8L) gebildet wurde, in Acetat und L-Aspartat zu spalten.
Tiran, Verena
core
Biochemical heterogeneity of infantile central nervous system spongy degeneration
, 2017 Aspartoacylase, the enzyme whose activity is deficient in infantile central nervous system spongy degeneration (Canavan-Van Bogaert-Bertrand disease), is detected as an approximately 59-kD protein in the Sephadex G-200 filtration of normal fibroblast ...
Youssef, Nazih +2 more
core +1 more source
The effect of N-acetyl-aspartyl-glutamate and N-acetyl-aspartate on white matter oligodendrocytes
, 2009 Elevations of the levels of N-acetyl-aspartyl-glutamate (NAAG) and N-acetyl-aspartate (NAA) are associated with myelin loss in the leucodystrophies Canavan's disease and Pelizaeus-Merzbacher-like disease. NAAG and NAA can activate and antagonize neuronal
Attwell, D. +9 more
core +1 more source