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Canavan Disease
Known as:
Disease, Canavan
, Leukodystrophy, Spongiform
, ASPA DEFICIENCY
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A rare neurodegenerative condition of infancy or childhood characterized by white matter vacuolization and demeylination that gives rise to a spongy…
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National Institutes of Health
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Related topics
Related topics
20 relations
ASPA gene
Aminoacylase 2 Deficiency
Aspartoacylase
Autosomal recessive inheritance
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Narrower (1)
Canavan Disease, Neonatal
Broader (1)
Leukodystrophy
Papers overview
Semantic Scholar uses AI to extract papers important to this topic.
Highly Cited
2014
Highly Cited
2014
A spongy graphene based bimorph actuator with ultra-large displacement towards biomimetic application.
Ying Hu
,
T. Lan
,
Guanyuan Wu
,
Zicai Zhu
,
Wei Chen
Nanoscale
2014
Corpus ID: 24047157
Bimorph actuators, consisting of two layers with asymmetric expansion and generating bending displacement, have been widely…
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Review
2007
Review
2007
Brain N-acetylaspartate as a molecular water pump and its role in the etiology of canavan disease
M. Baslow
Journal of Molecular Neuroscience
2007
Corpus ID: 12350717
N-acetyl-L-aspartate (NAA), an abundant amino acid present in the vertebrate brain, is synthesized and stored primarily in…
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Highly Cited
1996
Highly Cited
1996
Quantitative measurements with localized 1H MR spectroscopy in children with Canavan's disease
H. Wittsack
,
H. Kugel
,
B. Roth
,
W. Heindel
Journal of Magnetic Resonance Imaging
1996
Corpus ID: 45581019
Canavan's disease is an autosomal recessive hereditary leukodystrophy resulting from deficiency of the enzyme aspartoacylase. Two…
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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
Tutomu Amano
,
H. Nakanishi
,
M. Oka
,
Kenji Yamamoto
Experimental Neurology
1995
Corpus ID: 2896256
Senescence-accelerated mouse (SAM) P8 and P10 exhibit a spongy degeneration, especially in the brain stem, and a brain atrophy…
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Highly Cited
1992
Highly Cited
1992
Proton NMR spectroscopy of Canavan's disease.
P. Barker
,
R. Bryan
,
Ashok J. Kumar
,
S. Naidu
Neuropediatrics
1992
Corpus ID: 44466248
Proton Nuclear Magnetic Resonance (NMR) spectroscopy was used to quantitatively determine cerebral N-Acetyl Aspartate (NAA…
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1992
1992
Senecio spp poisoning in cattle in southern Brazil.
C. S. D. de Barros
,
D. Driemeier
,
C. Pilati
,
S. Barros
,
L. M. Castilhos
Veterinary and human toxicology
1992
Corpus ID: 24198108
Epidemiological, clinical, necropsy and histopathological data were accumulated during the study of 15 outbreaks of Senecio spp…
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Highly Cited
1989
Highly Cited
1989
Spontaneous Spongy Degeneration of the Brain Stem in SAM-P/8 Mice, a Newly Developed Memory-Deficient Strain
H. Yagi
,
M. Irino
,
+7 authors
T. Takeda
Journal of Neuropathology and Experimental…
1989
Corpus ID: 38403254
Abstract. A spontaneous spongy degeneration of the brain stem and spinal cord was discovered in a murine model of accelerated…
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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.
S. Rehm
,
P. Mehraein
,
Anzil Ap
,
F. Deerberg
Laboratory animal science
1982
Corpus ID: 41922598
A new spontaneous autosomal recessive mutation with a nervous disorder and a hair anomaly occurred in a colony of Sprague Dawley…
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Highly Cited
1972
Highly Cited
1972
Cerebral spongy degeneration of infancy
B. Adornato
,
J. O'brien
,
P. Lampert
,
T. Roe
,
H. Neustein
Neurology
1972
Corpus ID: 36750644
THE CLINICAL DEFINITION of van Bogaert-Bertrand spongy degeneration of infancy has been well established.1%2 Megalencephaly…
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Highly Cited
1966
Highly Cited
1966
FINE STRUCTURE OF SPONGY DEGENERATION OF THE CENTRAL NERVOUS SYSTEM (van BOGAERT AND BERTRAND TYPE)
M. Adachi
,
B. J. Wallace
,
L. Schneck
,
B. Volk
Journal of Neuropathology and Experimental…
1966
Corpus ID: 36198251
The present study describes the fine structure of cortex and white matter of a one year old Jewish boy afflicted with spongy…
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