Japanese
 NBRP Rat No: 0576 Strain Name: ALD/Hyo Commmon Name: Wolman's disease rat, Yoshida rat
 Principal Investigator  Hiroki Yoshida
 Organization   Kagoshima University President
 Address  1-21-24 Korimoto Kagoshima

890-8580 Kagoshima

  Japan
 Telephone  099-285-7000  Fax:  099-285-7001  gakucyo@kuas.kagoshima-u.ac.jp
 Inbred Generations   F40+? (May 2009) 
   
 Coat Color
 Deposition Status
 
 albino (c)
  Embryo      Sperm      Live Animals
 Usage Restrictions   
 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 Lipa: lipase A, lysosomal acid, cholesterol esterase
 Origin In a colony of Donryu rats, Yoshida found a male rat showing hepatomegaly and splenomegaly in 1981 (Yoshida, 1990). These mutant rats were transferred to Kagoshima University and maintained in heterozygous condition so far. ALD (acid lipase deficiency). (Mar 15, 2010) 
 Strain Characteristics ALD rats have congenital deficiency of lysosomal acid lipase. Affected rats markedly accumulate cholesteryl ester, free cholesterol and triglyceride in the liver, and cholesteryl esters and free cholesterol in the spleen (Kuriyama, 1990; Kuriwaki, 1999). ALD is a disease model for Wolman’s disease. <br />
ALD rat has a large deletion at the 3’-end of the </i>Lipa</i> gene (Nakagawa, 1995). (Mar 15, 2010) 
 Breeding Conditions Homozygous animals die until 120 days of age from liver failures. Homozygous ALD rats are infertile. Maintained in heterozygous condition. (Mar 15, 2010) 
 Genotyping Please refer to reference 3 (Nakagawa, 1995).
<a href="http://www.anim.med.kyoto-u.ac.jp/nbr/documents/PCR_Gene/Lich_en.pdf">Genotyping protocol for Lich</a> 
 References  1.Yoshida H, Kuriyama M.
Genetic lipid storage disease with lysosomal acid lipase deficiency in rats.
Lab Anim Sci. 1990 Sep;40(5):486-9.

2.Kuriyama M, Yoshida H, Suzuki M, Fujiyama J, Igata A.
Lysosomal acid lipase deficiency in rats: lipid analyses and lipase activities in liver and spleen
J Lipid Res. 1990 Sep;31(9):1605-12.

3.Nakagawa H, Matsubara S, Kuriyama M, Yoshidome H, Fujiyama J, Yoshida H, Osame M.
Cloning of rat lysosomal acid lipase cDNA and identification of the mutation in the rat model of Wolman's disease.
J Lipid Res. 1995 Oct;36(10):2212-8.

4.Kuriwaki K, Yoshida H.
Morphological characteristics of lipid accumulation in liver-constituting cells of acid lipase deficiency rats (Wolman's disease model rats).
Pathol Int. 1999 Apr;49(4):291-7.

5. Thelwall PE, Smith FE, Leavitt MC, Canty D, Hu W, Hollingsworth KG, Thoma C, Trenell MI, Taylor R, Rutkowski JV, Blamire AM, Quinn AG.
Hepatic Cholesteryl Ester Accumulation in Lysosomal Acid Lipase Deficiency: Non-invasive Identification and Treatment Monitoring by Magnetic Resonance.
J Hepatol. 2013 Sep;59(3):543-9.