Japanese
 NBRP Rat No: 0015 Strain Name: TRM/Kyo Commmon Name: TRM, tremor rat
 Principal Investigator  Tadao Serikawa
 Organization   Graduate School of Medicine, Kyoto University Institute of Laboratory Animals
 Address  Yoshidakonoe-cho, Sakyo-ku

606-8501 Kyoto

  JAPAN
 Telephone  075-753-4360  Fax:  075-753-4409  serikawa@anim.med.kyoto-u.ac.jp
 Inbred Generations   F69 (March 2012) 
   
 Coat Color
 Deposition Status
 
 albino (c)
  Embryo      Sperm      Live Animals
 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   Inbred   Segregating   Congenic   Consomic    Recombinant 
  Coisogenic   Spont. Mutant    Transgene   Ind. Mutant    Others 
 Comercial Availability   
 Research Category   Diabetes Obesity    Neurobiology    Ophthalmology    Dentistry    Cardio- Hypertension 
  Oncology   Metabolism   Otorhinology    Immunology    Infectious Disease
  Osteology    Internal Medicine   Dermatology   Reproduction    Development
  Behavior    Hematology    Urology   Pharmacology   Others 
  Control Strains   Reporter gene Strains  
 Gene 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 (<i>tm</i>). TRM was established as a segregating inbred strain. In F18 progeny, a rat which did not have <i>tm</i> 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 <i>tm</i>/<i>tm</i> rats and at about 26 weeks of age in <i>tm</i>/+ 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 (<i>Aspa</i>), 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 <i>Trpv3</i> and other genes. (Dec 8, 2010) 
 Breeding Conditions Homozygous infertility. Maintained in heterozygous condition. 
 Genotyping Genomic deletion of Aspa gene is detected by PCR.
<a href="http://www.anim.med.kyoto-u.ac.jp/nbr/documents/PCR_Gene/tm_en.pdf">Genotyping protocol for tm</a> 
 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.