Page last updated: 2024-10-18

glutaric acid and Canavan Disease

glutaric acid has been researched along with Canavan Disease in 3 studies

glutaric acid: RN given refers to parent cpd
glutaric acid : An alpha,omega-dicarboxylic acid that is a linear five-carbon dicarboxylic acid.

Canavan Disease: A rare neurodegenerative condition of infancy or childhood characterized by white matter vacuolization and demeylination that gives rise to a spongy appearance. Aspartoacylase deficiency leads to an accumulation of N-acetylaspartate in astrocytes. Inheritance may be autosomal recessive or the illness may occur sporadically. This illness occurs more frequently in individuals of Ashkenazic Jewish descent. The neonatal form features the onset of hypotonia and lethargy at birth, rapidly progressing to coma and death. The infantile form features developmental delay, DYSKINESIAS, hypotonia, spasticity, blindness, and megalencephaly. The juvenile form is characterized by ATAXIA; OPTIC ATROPHY; and DEMENTIA. (From Adams et al., Principles of Neurology, 6th ed, p944; Am J Med Genet 1988 Feb;29(2):463-71)

Research Excerpts

ExcerptRelevanceReference
"We report three cases of L-2-hydroxyglutaric acidemia and three cases of Canavan disease."7.69Clinical and magnetic resonance imaging features of L-2-hydroxyglutaric acidemia: report of three cases in comparison with Canavan disease. ( Aktan, G; Erdem, G; Jacobs, C; Renda, Y; Saatçi, I; Schutgens, RB; Simşek, A; Topçu, M; Wanders, RJ, 1996)
"We report three cases of L-2-hydroxyglutaric acidemia and three cases of Canavan disease."3.69Clinical and magnetic resonance imaging features of L-2-hydroxyglutaric acidemia: report of three cases in comparison with Canavan disease. ( Aktan, G; Erdem, G; Jacobs, C; Renda, Y; Saatçi, I; Schutgens, RB; Simşek, A; Topçu, M; Wanders, RJ, 1996)

Research

Studies (3)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's1 (33.33)18.2507
2000's2 (66.67)29.6817
2010's0 (0.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Yamaguchi, S1
Bal, D1
Kraska-Dziadecka, A1
Gradowska, W1
Gryff-Keller, A1
Topçu, M1
Erdem, G1
Saatçi, I1
Aktan, G1
Simşek, A1
Renda, Y1
Schutgens, RB1
Wanders, RJ1
Jacobs, C1

Reviews

1 review available for glutaric acid and Canavan Disease

ArticleYear
[Organic acid disorders: cerebral organic acidemia].
    Ryoikibetsu shokogun shirizu, 2002, Issue:37 Pt 6

    Topics: Brain Diseases, Metabolic; Canavan Disease; Glutarates; Glycerol; Humans; Hydroxybutyrates; Infant;

2002

Other Studies

2 other studies available for glutaric acid and Canavan Disease

ArticleYear
Investigation of a wide spectrum of inherited metabolic disorders by 13C NMR spectroscopy.
    Acta biochimica Polonica, 2008, Volume: 55, Issue:1

    Topics: Biomarkers; Canavan Disease; Glutarates; Hemiterpenes; Humans; Lactic Acid; Magnetic Resonance Spect

2008
Clinical and magnetic resonance imaging features of L-2-hydroxyglutaric acidemia: report of three cases in comparison with Canavan disease.
    Journal of child neurology, 1996, Volume: 11, Issue:5

    Topics: Canavan Disease; Cerebellar Nuclei; Child; Child, Preschool; Female; Glutarates; Humans; Magnetic Re

1996