- N-Acetylaspartate Synthase Deficiency Corrects the Myelin Phenotype in a Canavan Disease Mouse Model But Does Not Affect Survival Time.
- Maier H1, Wang-Eckhardt L1, Hartmann D2, Gieselmann V3, Eckhardt M4.
- The Journal of neuroscience : the official journal of the Society for Neuroscience.J Neurosci.2015 Oct 28;35(43):14501-16. doi: 10.1523/JNEUROSCI.1056-15.2015.
- Canavan disease (CD) is a severe, lethal leukodystrophy caused by deficiency in aspartoacylase (ASPA), which hydrolyzes N-acetylaspartate (NAA). In the brains of CD patients, NAA accumulates to high millimolar concentrations. The pathology of the disease is characterized by loss of oligodendrocytes
- PMID 26511242
- Leukodystrophy with multiple beaded periventricular cysts: unusual cranial MRI results in Canavan disease.
- Drenckhahn A1, Schuelke M, Knierim E.
- Journal of inherited metabolic disease.J Inherit Metab Dis.2015 Sep;38(5):983-4. doi: 10.1007/s10545-015-9812-1. Epub 2015 Feb 3.
- A 3-year-old boy was admitted with psychomotor delay, spasticity, progressive visual loss, nystagmus, macrocephaly, and epileptic seizures for diagnostics. Cranial magnetic resonance imaging (MRI) revealed leukodystrophy and multicystic changes. Urine excretion of N-acetylaspartic acid was grossly i
- PMID 25647544
- Frontiers of Model Animals for Neuroscience: Two Prosperous Aging Model Animals for Promoting Neuroscience Research
- ITO Koichi
- Experimental Animals 62(4), 275-280, 2013
- … SAMP8 has spontaneous spongy degeneration in the brain stem and spinal cord with aging, and immunohistochemical studies reveal excess protein expression of amyloid precursor protein and amyloid β in the brain, indicating that SAMP8 is a model for Alzheimer's disease. …
- NAID 130003382710
- Hexachlorophene and Cuprizone Induce the Spongy Change of the Developing Rat Brain by Different Mechanisms: The Role of 2', 3'-Cyclic Nucleotide 3'-Phosphodiesterase (CNPase)
- KANNO Takeshi,SASAKI Satoshi,YAMADA Naoaki [他],KAWASAKO Kazufumi,TSUCHITANI Minoru
- Journal of Veterinary Medical Science 74(7), 837-843, 2012
- … The goal of this research was to identify mechanisms responsible for the spongy change induced in rats after repeated hexachlorophene (HCP) or cuprizone (CPZ) dosing. … Rats were dosed with 35 mg/kg HCP for 5 days followed by drug withdrawal for 7 days suffered spongy changes to the white matter of the cerebrum, cerebellum, medulla oblongata, and spinal cord that were accompanied by degeneration of oligodendroglia. …
- NAID 130001879771
- degeneracy、degenerate、degenerative、denaturation、denature、involution、retract、retrograde degeneration、retrograde degradation、unfolding