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Genetic Status
Inbred
Segregating
Congenic
Consomic
Recombinant
Coisogenic
Spont. Mutant
Transgene
Ind. Mutant
Category Other
Comercial Availability
Research Category
Diabetes Obesity
Neurobiology
Ophthalmology
Dentistry
Cardio Hypertension
Cancer
Metabolism
Otorhinology
Immunology
Infectious
Osteosis
Internal Organ
Dermatology
Reproduction
Development
Behavior
Hematology
Urology
Pharmacology
Research Area Others
Control Strain
Marker Strain
Gene Affected
Aspa: aspartoacylase
Origin
Tremor Rat 1980 was found in a colony of outbred Wistar/Kyo while establishing inbred strains from Wistar/Kyo by spontanious mutation. After F18, a control line WTC was separated.
Strain characteristics
epilepsy, absence-like seizures, vacuole formation. Segregating inbred strain. The tremor rat has a large deletion (200kb) spanning the aspartoacylase (ASPA) coding region and thus represents a naturally occurring model of Canavan disease. The deleted resion includes several other genes such as Trpv3, which is known to be related with abnormal hair.
Breeding Conditions
Homozygous infertility. Maintained in heterozygous condition.
Genotyping
Genomic deletion of Aspa gene is detected by PCR.
References
Yamada J, Serikawa T, Ishiko J, Inui T, Takada H, Kawai Y and Okaniwa, A.
Rats with congenital tremor and curled whiskers and hair.
Jikken Dobutsu, 34: 183-188, 1985.
Serikawa T, Ohno Y, Sasa M, Yamada J and Takaori, S.
A new model of petit mal epilepsy: spontaneous spike and wave discharges in tremor rats.
Lab Anim, 21: 68-71, 1987.
Kondo A, Nagara H, Akazawa K, Tateishi J, Serikawa T and Yamada, J.
CNS Pathology in the neurological mutant rats zitter, tremor and zitter-tremor double mutant (spontaneously epileptic rat, SER).
Brain, 114, 979-999, 1991.
Kitada K, Akimitsu T, Shigematsu Y, Kondo A, Maihara T, Yokoi N, Kuramoto T, Sasa M and Serikawa, T.
Accumulation of N-acetyl-L-aspartate in the brain of the tremor rat, a mutant exhibiting absence-like seizure and spongiform degeneration in the central nervous system.
J Neurochem, 74: 2512-2519, 2000.
Asakawa M, Yoshioka T, Matsutani T, Hikita I, Suzuki M, Oshima I, Tsukahara K, Arimura A, Horikawa T, Hirasawa T, Sakata T.
Association of a mutation in TRPV3 with defective hair growth in rodents.
J Invest Dermatol. 126(12):2664-72, 2006.
Wang J, Leone P, Wu G, Francis JS, Li H, Jain MR, Serikawa T, Ledeen RW.
Myelin Lipid Abnormalities in the Aspartoacylase-Deficient Tremor Rat.
Neurochem Res. 2009 Jan;34(1):138-48.
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Inbred