Rat Strains

Principal Investigator:

Tadao Serikawa Graduate School of Medicine, Kyoto University Yoshidakonoe-cho, Sakyo-ku 606-8501 Kyoto JAPAN

Preservation Status:

Embryo Sperm Living Animals

Coat Color

albino (c)

Inbred Generations

F69 (March 2012)

Usage Restrictions

The recipient of BIOLOGICAL RESOURCE shall obtain a prior written consent on use of it from the DEPOSITOR.
For a commercial use of this resource, a new contract must be concluded between the depositor and the recipient.

Genetic Status

Comercial Availability

Research Category

Gene Affected

Aspa: aspartoacylase

Origin

In 1980, a spontaneous tremor mutant rat was found in the colony of Kyo:Wistar (Yamada, 1985). This disorder was found to be caused by an autosomal recessive gene and was designated tremor (tm). TRM was established as a segregating inbred strain. In F18 progeny, a rat which did not have tm mutation was separated and established as a control strain of WTC (NBRP No.0020). (Dec 8, 2010)

Strain characteristics

TRM develops tremor of the whole body at 2 weeks of age and the tremor gradually disappears at 6-8 weeks of age (Yamada, 1985). 5-7 spike-and-wave complex associated with behavioral absence-like seizure starts at about 14 weeks of age in tm/tm rats and at about 26 weeks of age in tm/+ rats (Serikawa,1987 and Higashiguchi, 1991). TRM shows vacuole formation in the CNS, aplastic gonads in both sexes (Yamada, 1985), and auditory deficit (Kuse, 1991). By positional cloning, a genomic deletion more than 200-kb was found within the critical region in which the aspartoacylase gene (Aspa), the causative gene for the human Canavan disease, is located (Kitada, 2000). Accumulation of NAA is observed especially in pons and thalamus and it would induce their disease state. The deleted region also contains Trpv3 and other genes. (Dec 8, 2010)

Breeding Conditions

Homozygous infertility. Maintained in heterozygous condition.

Genotyping

Genomic deletion of Aspa gene is detected by PCR. Genotyping protocol for tm

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.

Klugmann M1, Leichtlein CB, Symes CW, Serikawa T, Young D, During MJ.
Restoration of aspartoacylase activity in CNS neurons does not ameliorate motor deficits and demyelination in a model of Canavan disease.
Mol Ther. 2005 May;11(5):745-53.

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.

Arun P, Madhavarao CN, Moffett JR, Hamilton K, Grunberg NE, Ariyannur PS, Gahl WA, Anikster Y, Mog S, Hallows WC, Denu JM, Namboodiri AM.
Metabolic acetate therapy improves phenotype in the tremor rat model of Canavan disease.
J Inherit Metab Dis. 2010 Jun;33(3):195-210.

Serikawa T, Mashimo T, Kuramoto T, Voigt B, Ohno Y, Sasa M.
Advances on Genetic Rat Models of Epilepsy.
Exp Anim. 64(1):1-7. 2015.

Ohno Y, Shimizu S, Tatara A, Imaoku T, Ishii T, Sasa M, Serikawa T, Kuramoto T.
Hcn1 is a tremorgenic genetic component in a rat model of essential tremor.
PLoS One. 2015 May 13;10(5):e0123529.

Kuwamura M, Tanimura S, Hasegawa Y, Hoshiai R, Moriyama Y, Tanaka M, Takenaka S, Nagayoshi H, Izawa T, Yamate J, Kuramoto T, Serikawa T.
Downregulation of aspartoacylase during the progression of myelin breakdown in the dmy mutant rat with mitochondrial magnesium channel MRS2 defect.
Brain Res. 2019 Sep 1:1718:169-175.

Additional strain information

TRM