Strain information
 NBRP Rat No: 0556  Strain name: AR-EdnrbSl/Okkm  Commmon Name: Aganglionosis rat Rat Genome Database
Principal Investigator:  Tohru Kimura  National Institute for Physiological Sciences       38 Nishigo-naka, Myodaiji, Okazaki    444-8585 Aichi     Japan
Tel: 0564-55-7882    Fax: 0564-55-7881 Email:
Preservation Status:   Embryo        Sperm       Living Animals
Coat Color  black hooded
Inbred Generations  
Usage Restrictions  In publishing, an acknowledgment to the DEPOSITOR is requested.
Genetic Status
 Inbred  Segregating  Congenic  Consomic  Recombinant
 Coisogenic  Spont. Mutant  Transgene  Ind. Mutant  Category Other closed colony
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 Ednrb: endothelin receptor type B
Origin Congenital megacolon rats were found in offspring of a female albino rat crossed with a wild male by Ikadai et al. at Institute for Animal Reproduction in 1973 and were named Aganglionosis Rat (AR) (Ikadai, 1979). (Jul 7, 2009)
Strain characteristics AR rats are characterized by megacolon and white coat color with a small pigmented spot on the head (Ikadai, 1979). AR shows a long constricted segment of the intestine, which extended from the distal ileum to the rectum. Histologically, myenteric and submucosal plexus are absent. AR is considered to be an animal model of Hirshsprung’s disease. AR dies of an intestinal obstruction from 1 to 5 weeks of age. The autosomal recessive spotting lethal, sl, was revealed to be caused by a 301-bp interstitial deletion of Endothelin B receptor (Ednrb) (Ceccherini, 1995). (Jul 7, 2009)
Breeding Conditions Homozygous sl rats die within 1-5 weeks of age. The sl mutation is maintained in heterozygous condition (closed colony). (Jul 7, 2009)
Genotyping Genotyping protocol for Ednrb
References 筏井洋、他
先天性 Aganglionosis rat (Hirschsprung 病rat) の発見とその遺伝学的研究
先天異常 19: 31-36, 1979
Horie, H., Ikadai, H., Iwasaki, I., Ide, G., Takahashi, H.
Pathologic studies on newly established cingenital aganglionosis rat in Japan.
日本小児外科学会雑誌 16: 27-38, 1980
Ikadai, H., Shimizu, K., Nakajyo, S., Imamichi, T., Sasaki, Y., Urakawa, N.
Responses of narrow segments of intestines in the congenital aganglionosis rat to various stimulants.
Nippon Heikatsukin Gakkai Zasshi. 18: 347-361, 1982
Horisawa, M., Isiguro, Y., Ito, T., Kato, K.
Distribution of nervous system-specific enolase (NSE) and S-100 protein in the gastrointestinal tract of congenital aganglionosis rat.
Nippon Heikatsukin Gakkai Zasshi. 21: 11-24, 1985
Nagahama, M., Ozaki, T., Hama, K.
A study of the myenteric plexus of the congenitalaganglionosis rat (spotting lethal).
Anat. Embryol. 171: 285-96, 1985
Goto, S., Gunter, M., Scherer, LR., Bloch, T., Grosfeld, JL.
Surgical treatment of total colonic aganglionosis: efficacy of aganglionic patch enteroplasty in the rat.
J. Ped. Surgery. 21: 601-607, 1986
Domoto, T., Oki, M., Gonda, T., Inoue, K., Ozaki, T.
Distribution of nerves containing vasoactive intestinal polypeptide-like immunoreactivity in rats with congenital aganglionosis of the colon.
Neurosci. Res. 4: 309-315, 1987
Hirose, R., Nada, O., Kawana,T., Goto, S., Taguchi, T., Toyohara, T., Ikeda, K.
An immunohistochemical study of somatostatin-containing nerves in the aganglionic colon of human and rat.
Acta Neuropathol. 78: 372-379, 1989.
Gonda, T. Oki, M.
Distribution of cholinergic and catecholaminergic nerves in the colon of the rat with aganglionosis.
Jikken Dobutsu. Exp. Anim. 40: 471-484, 1991
Hirose, R., Nada, O., Kawana, T., Suita, S.
The intramural pelvic nerves immunoreactive for calcitonin gene-relatedpeptide in the rectum of normal and aganglionosis rat.
Anatom. Embryol. 187: 37-44, 1993
Nagahama, M., Tsutsui, Y., Ozaki, T., Hama, K.
Myenteric and submucosal plexuses of the congenital aganglionosis rat (spotting lethal) as revealed by scanning electron microscopy.
Biol. Signals. 2: 136-145, 1993
Domoto, T., Zhang, WB., Tsumori, T., Oki, M.
Distribution of extrinsic enkephalin-containing nerve fibers in the rat rectum and their origin in the major pelvic ganglion.
J. Aut. Nerv. Sys. 49: 135-146, 1994
Ceccherini, I., Zhang, AL., Matera, I., Yang, G., Devoto, M., Romeo, G., Cass, DT.
Interstitial deletion of the endothelin-B receptor gene in the spotting lethal (sl) rat
Hum. Mol. Genet. 4: 2089-2096, 1995
Yanai, T., Yamataka, A., Nagaoka,I., Fujimoto, T., Nishiye, H., Yamashita, T., Miyano, T.
Quantitative analysis of synaptic vesicle proteins in the aganglionic colon in rats with congenital aganglionosis.
J. Ped. Gastroenterol Nutr. 20: 239-240, 1995
Watanabe, Y., Ito, T., Harada, T., Takahashi, M., Kobayashi, S., Ozaki, T., Nimura, Y.
Expression of ret proto-oncogene products in the hypoganglionic segment of the small intestine of congenital aganglionosis rats.
J. Ped. Surgery. 30: 641-645, 1995
Watanabe, Y., Ito, T., Harada, T., Kobayashi, S., Ozaki, T., Nimura, Y.
Spatial distribution and pattern of extrinsic nerve strands in the aganglionic segment of congenital aganglionosis: stereoscopic analysis in spotting lethal rats.
J. Ped. Surgery. 30: 1471-1476, 1995
Teramoto, M., Domoto, T., Tanigawa, K., Yasui, Y., Tamura, K.
Distribution of nitric oxide synthase-containing nerves in the aganglionic intestine of mutant rats: a histochemical study.
J Gastroenterol. 31: 214-223, 1996
Gariepy, CE., Cass, DT., Yanagisawa, M
Null mutation of endothelin receptor type B gene in spotting lethal rats causes aganglionic megacolon and white coat color.
Proc. Natl. Acad. Sci. USA. 93: 867-72, 1996
Gariepy, CE., Williams, SC., Richardson, JA., Hammer, RE., Yanagisawa, M.
Transgenic expression of the endothelin-B receptor prevents congenital intestinal aganglionosis in a rat model of Hirschsprung disease.
J. Clin. Invest. 102: 1092-101, 1998
Karaki, H., Mitsui-Saito, M., Takimoto, M., Oda, K., Okada, T., Ozaki, T., Kunieda, T.
Lack of endothelin ETB receptor binding and function in the rat with a mutant ETB receptor gene.
Biochem. Biophys. Res. Commun. 222: 139-143, 1996
Kunieda, T., Kumagai, T., Tsuji, T., Ozaki, T., Karaki, H., Ikadai, H.
A mutation in endothelin-B receptor gene causes myenteric aganglionosis and coat color spotting in rats.
DNA Res. 3: 101-105, 1996
Watanabe, Y., Ito, T., Iino, S., Harada, T., Ando, H., Seo, T., Kobayashi, S., Ozaki T.
Unique spindle cells in the involved ileal segment of rats with congenital aganglionosis.
J. Ped. Surgery. 32: 1229-1233, 1997
Gariepy, CE., Williams, SC., Richardson, JA., Hammer, RE., Yanagisawa, M.
Transgenic expression of the endothelin-B receptor prevents congenital intestinal aganglionosis in a rat model of Hirschsprung disease.
J. Clin. Invest. 102:1092-1101, 1998
Matsumura, Y., Kuro, T., Kobayashi, Y., Konishi, F., Takaoka, M., Wessale, JL., Opgenorth, TJ., Gariepy, CE., Yanagisawa M.
Exaggerated vascular and renal pathology in endothelin-B receptor-deficient rats with deoxycorticosterone acetate-salt hypertension.
Circulation 102: 2765-2773, 2000

Additional strain information AR (Aganglionosis rat)