• Search Research Projects
  • Search Researchers
  • How to Use
  1. Back to previous page

ESTABLISHMENT OF A RAT STRATIN WITH MUCOPOLYSUCCHARIDOSIS TYPE VI AS AN ANIMAL MODEL FOR GENE THERAPY

Research Project

Project/Area Number 07558240
Research Category

Grant-in-Aid for Scientific Research (A)

Allocation TypeSingle-year Grants
Section試験
Research Field Laboratory animal science
Research InstitutionOKAYAMA UNIVERSITY

Principal Investigator

KUNIEDA Tetsuo  OKAYAMA UNIVERSITY,FACULTY OF AGRICULTURE,ASSOCIATE PROFESSOR, 農学部, 助教授 (80178011)

Co-Investigator(Kenkyū-buntansha) AMAO Hiromi  NIPPON VETERINARY AND AMINAL SCIENCE UNIVIERSITY,DEPARTMENT OF LABOLATORY ANIMAL, 獣医畜産学部, 講師 (60151114)
WAKAFUJI Yasumasa  IMAMICHI INSTITUTE FOR ANIMAL REPRODUCTION., 副主任研究員 (80158592)
YOSHIDA Midori  SASAKI INSTITUTE,DEPARTMENT OF PATHOLOGY., 病理部, 研究助手 (70201861)
SAKIYAMA Takeshi  ST MARIANNA UNIVERSITY SCHOOL OF MEDICINE,FIRST DEPARTMENT OF PATHOLOGY,ASSOCIAT, 医学部, 助教授 (20130510)
Project Period (FY) 1995 – 1996
Project Status Completed (Fiscal Year 1996)
Budget Amount *help
¥1,900,000 (Direct Cost: ¥1,900,000)
Fiscal Year 1996: ¥1,900,000 (Direct Cost: ¥1,900,000)
KeywordsRATS / ANIMAL MODEL / MUCOPOLYSUCCHARIDOSIS / LYSOSOME / ARYLSULFATASE / MUTATION / SOMATIC-CELL HYBRIDS / MAPPING / ワソゾーム
Research Abstract

The present research project was carried out to establish the MPR rat as an animal model for human hereditary mucopolysuccharidosis type VI by identifying the mutation responsible for the phenotypes and by examinating therapeutic effects of bone marrow transplantation.
We compaired the nucleotide sequences of the rat arylsulfatase B gene by cloning nad sequencing the gene, and found an insertional mutation that causes a fram-shift of the gene. We further revealed that an amplification of the region including the mutation enables to easily identify carriar rats of the mutation.
We performed bone marrow transplantation to confirm therapeutic effects of supplement of the normal enzyme producing cells. The recipient rats showed no accumulation of glycosaminoglycan in organs and no urinary excretion of the glycosaminoglycan but partial resoluation of facial dysmorphia and bony feutures, indicating therapeutic effects of the bone marrow transplantation.
We, therefore, concluded that the MPR rat is a useful animal model for mucopolysaccharidosis type VI of human.

Report

(3 results)
  • 1996 Annual Research Report   Final Research Report Summary
  • 1995 Annual Research Report
  • Research Products

    (18 results)

All Other

All Publications (18 results)

  • [Publications] Kuniede et al: "Mucopolysucharidosis type VI in rats." Genomics. 29. 582-587 (1995)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Kunieda et al: "A mutation in endothelin-Breceptor gene…" DNA Res.3. 101-105 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Karaki et al.: "Lack of endothelin ET Breceptor binding…" Biochem.Biophys. Res.Commun. 222. 139-143 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Kobayashi et al: "Localization of Na^+,K^+、ATPase a snbmnitgone" Mamm. Genom. 6. 889-889 (1995)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Kunieda et al: "Localization of Moloney murine lenkemia…" Mamm-Genom.7. 924-925 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Yoshida et al: "A case report of snperficial necrolytic…" Toxical.Pathol. 24. 498-501 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Kunieda, T., Simonaro, C.M., Yoshida, M., Ikadai, H., Levan, G., Desnick, R.J., and Schuchman, E.H.: "Mucopolysaccharidosis type VI in rats : Isolation of cDNA encoding arylsulfataseB,chromosomal localizaiton of the gene, and identification of the mutation." Genomics. 29. 582-587 (1995)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Kunieda, T., Kumagai, T., Tsuji, T., Ozaki, T., Karaki, H., and Ikadai, H.: "A mutation in endothelin-B receptor gene causes myenteric aganglionosis and coat color spotting in rats." DNA Res.3. 101-105 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Karaki, H., Mitui-Saito, M., Takimoto M., Oda, K., Okada, T., Ozaki, T., and Kunieda, T.: "Lack of endothelin ETB receptor binding and funciton in the rat with a mutant ETB receptor gene." Biochem.Biophys.Res.Commun.222. 139-143 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Kobayashi, E., Tachibana, M., Ikadai, H., and kunieda, T.: "Localization of Na^+, K^+-ATPase a 2 subunit gene, ATP1A2, on rat chromosome 13." Mamm.Genom. 6. 889 (1995)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Kunieda, T., Kobayashi, E., Tachibana, M., Ikadai, H.: "Localizaion of a Moloney murine leukemia virus integration site gene, M1vi2, on rat chromosome 2." Mamm.Genom.7. 924-925 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Yoshida M,Barata K,Takahashi M Ando-Lu J and Maekawa A.: "A case report of superficial necrolytic dermatitis in a beagle dog with diabetes mellitus." Toxicol.Pathol.24. 498-501 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] T.Kunieda et al: "A muctation in endothelin-B receptor geve causes ・・・・" DNA Research. 3. 101-105 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] H.Karaki et al: "Lack of endothelin ETB receptor binding and ・・・・" Biochem.Biophys.Res.Commun.222. 139-143 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] R.Valdivia et al: "Typing of X chromosome bearing Tabby allele in ・・・・" Eyp.Anim. 45. 395-398 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] T.Kunieda et al: "Localization of a Moloney murine Leukemia virus ・・・・" Mamm.Genome. 7. 924-925 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] 国枝哲夫: "牛における遺伝性疾患の分子遺伝学的解析" 獣医畜産新報 J.V.M.49. 413-416 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] Kunieda, T: "Mucopolysacchridosis Type VI in Rats." Genomics. 29. 582-587 (1995)

    • Related Report
      1995 Annual Research Report

URL: 

Published: 1996-04-01   Modified: 2016-04-21  

Information User Guide FAQ News Terms of Use Attribution of KAKENHI

Powered by NII kakenhi