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Canavan Disease

Known as: Disease, Canavan, Leukodystrophy, Spongiform, ASPA DEFICIENCY 
A rare neurodegenerative condition of infancy or childhood characterized by white matter vacuolization and demeylination that gives rise to a spongy… 
National Institutes of Health

Related topics

Related topics

20 relations
ASPA geneAminoacylase 2 DeficiencyAspartoacylaseAutosomal recessive inheritance

Narrower (1)

Canavan Disease, Neonatal

Broader (1)

Leukodystrophy

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
2007
2007
Expression of Iba1 protein in microglial cells of zitter mutant rat
1999
1999
The spectrum of mutations of the aspartoacylase gene in Canavan disease in non-Jewish patients
Canavan disease is an infantile neurodegenerative disease that is caused by mutations in the gene encoding the enzyme… 
1995
1995
Increased Expression of Cathepsins E and D in Reactive Microglial Cells Associated with Spongiform Degeneration in the Brain Stem of Senescence-Accelerated Mouse
Senescence-accelerated mouse (SAM) P8 and P10 exhibit a spongy degeneration, especially in the brain stem, and a brain atrophy… 
1993
1993
Management of Digestive and Liver Disorders in Infants and Children
Highly Cited
1992
Highly Cited
1992
Proton NMR spectroscopy of Canavan's disease.
Proton Nuclear Magnetic Resonance (NMR) spectroscopy was used to quantitatively determine cerebral N-Acetyl Aspartate (NAA… 
1991
1991
CNS pathology in the neurological mutant rats zitter, tremor and zitter-tremor double mutant (spontaneously epileptic rat, SER). Exaggeration of clinical and neuropathological phenotypes in SER.
The pathological alterations in the central nervous system (CNS) were examined in three kinds of mutant rat; the zitter (zi/zi… 
Review
1990
Review
1990
CT and MR imaging of Canavan disease.
We report here the MR findings (with CT correlation) in two siblings with Canavan disease and offer a review of the literature 
Highly Cited
1989
Highly Cited
1989
Spontaneous Spongy Degeneration of the Brain Stem in SAM-P/8 Mice, a Newly Developed Memory-Deficient Strain
Abstract. A spontaneous spongy degeneration of the brain stem and spinal cord was discovered in a murine model of accelerated… 
Highly Cited
1982
Highly Cited
1982
A new rat mutant with defective overhairs and spongy degeneration of the central nervous system: clinical and pathologic studies.
A new spontaneous autosomal recessive mutation with a nervous disorder and a hair anomaly occurred in a colony of Sprague Dawley… 
1968
1968
Protracted form of spongy degeneration of the central nervous system: (van Bogaert and Bertrand type)
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