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

2003 Fiscal Year Final Research Report Summary

GENE THERAPY FOR X-LINKED SEVERE COMBINED IMMUNODEFICIENCY DISEASE

Research Project

Project/Area Number 14370240
Research Category

Grant-in-Aid for Scientific Research (B)

Allocation TypeSingle-year Grants
Section一般
Research Field Pediatrics
Research InstitutionTOHOKU UNIVERSITY

Principal Investigator

TSUCHIYA Shigeru  TOHOKU UNIVERSITY, INSUTITUTE OF AGING, DEVELOPMENT AND CANCR, PROFESSOR, 加齢医学研究所, 教授 (30124605)

Co-Investigator(Kenkyū-buntansha) ANAN Kazuaki  TOHOKU UNIVERSITY, INSTITUTE OF AGING, DEVELPMENT AND CANCERAND CANCER, ASSISTANT, 加齢医学研究所, 助手 (80343036)
MINEGISHI Masayoshi  TOHOKU UNIVERSITY HOSPITAL, ASSOCIATE PROFESSOR, 医学部附属病院, 助教授 (20211592)
KUMAKI Satoru  MTOHOKU UNIVERSITY, INSTITUTE OF AGING, DEVELOPMENT AND CANCER, ASSOCIATE PROFESSOR, 加齢医学研究所, 助教授 (20311566)
Project Period (FY) 2002 – 2003
KeywordsX-linked Severe combined immunodeficiency / gene therapy / hematopoietic stem cell transplantation / non-myeloablative / haplotype identical / IL-'7receptor deficiency disease
Research Abstract

(1) Gene therapy for X-linked severe combined immunodeficiency (X-SCID)
Since two cases of leukemia like disease were reported from A Fischer's group among 11patients who received X-SCID gene therapy, the plan was now kept in suspense. We are going to reconsider the X-SCID gene therapy with new indications of the therapy.
(2) HLA haplotype identical peripheral stem cell transplantation to a patient with X-SCID
CD34 purified peripheral stem cell transplantation from the HLA haplotype identical mother were successfully carried out to the patient with XSCID and put on follow up study of his immune function. His immune function recovered soon without any evidence of graft versus host disease.
(3) Non-myeloablative bone marrow transplantation (NST) to a patient with chronic granulomatous diesesase(CGD)
NST was successfully performed to an 18-year-old patient with CGD. Chimerism of lymphocytes and granylocytes were achieved soon with recovery of superoxide production in granulocytes.
Combination of these two transplantation methods will enable us to establish more safe and efficient tranplantation method for X-SCID,
(4) IL-7receptor(R) α chain deficiency disease
We found IL-7R α deficiency disease among patients with SCID with B lymphocytes, the first case in asia.

  • Research Products

    (11 results)

All Other

All Publications (11 results)

  • [Publications] Itoh T et al.: "A simple controlled-rate freezing method without a rate-controlled programmed freezer provides optimal conditions for both large-scale and small-scale cryopreservation of umbilical cord blood cells"Transfusion. 43. 1303-1308 (2003)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Kobayashi N et al.: "Novel Artemis gene mutations of radiosensitive severe combined immunodeficiency in Japanese families."Hum Genet.. 112. 348-352 (2003)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Imashuku S et al.: "Multi-institutional collaborative study in Japan. Lack of clinical utility of minimal residual disease detection in allogeneic stem cell recipients with childhood acute lymphoblastic leukemia : multi-institutional collaborative study in Japan"Bone Marrow Transplant. 31. 1127-1135 (2003)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Kimura H et al.: "Prognostic factors for chronic active Epstein-Barr virus infection."J Infect Dis. 187. 527-533 (2003)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Kudo Y et al.: "The absolute number of peripheral blood 0034+ cells predicts a timing for apheresis and progenitor cell yield in patients with hematologic malignancies and solid tumors"Tohoku J Exp Med. 199. 111-118 (2003)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Itoh T, Minegishi M, Fushimi J, Takahashi H, Kudo Y, Suzuki A, Narita A, Sato Y, Akagi K, Wada Y, Saito A, Kikuchi M, Okamura K, Kaku M, Tsuchiya S.: "A simple controlled-rate freezing method without a rate-controlled programmed freezer provides optimal conditions for both large-scale and small-scale cryopreservation of umbilical cord blood cells"Transfusion. 43. 1303-1308 (2003)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Kobayashi N, Agematsu K, Sugita K, Sako M, Nonoyama S, Yachie A, Kumaki S, Tsuchiya S, Ochs HD, Sugita K, Fukushima Y, Komiyama A: "Novel Artemis gene mutations of radiosensitive severe combined immunodeficiency in Japanese families"Hum Genet.. 112. 348-352 (2003)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Imashuku S, Terui K, Matsuyama T, Asami K, Tsuchiya S, Ishii E, Kawa K, Kosaka Y, Eguchi H, Tsuchida M, Ikuta K, Kato S, Koizumi S, Okamura J, Morimoto A, Hibi S, Hamaoka K: "Multi-institutional collaborative study in Japan. Lack of clinical utility of minimal residual disease detection in allogeneic stem cell recipients with childhood acute lymphoblastic leukemia : multi-institutional collaborative study in Japan"Bone Marrow Transplant. 31. 1127-1135 (2003)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Kimura H, Morishima T, Kanegane H, Ohga S, Hoshino Y, Maeda A, Imai S, Okano M, Morio T, Yokota S, Tsuchiya S, Yachie A, Imashuku S, Kawa K, Wakiguchi H: "Japanese Association for Research on Epstein-Barr Virus and Related Diseases : Prognostic factors for chronic active Epstein-Barr virus infection"J Infect Dis. 187. 527-533 (2003)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Kimura H, Morishima T, Kanegane H, Ohga S, Hoshino Y, Maeda A, Imai S, Okano M, Morio T, Yokota S, Tsuchiya S, Yachie A, Imashuku S, Kawa K, Wakiguchi H: "Japanese Association for Research on Epstein-Barr Virus and Related Diseases : Prognostic factors for chronic active Epstein-Barr virus infection"J Infect Dis. 118. 313-319 (2002)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Kudo Y, Minegishi M, Saito N, Itoh T, Fushimi J, Takahashi H, Suzuki A, Narita A, Sato Y, Miura J, Yoshihara K, Meguro K, Imaizumi M, Sato M, Murakawa Y, Tsuchiya S.: "The Absolute Number of Peripheral Blood CD34+ Cells Predicts a Timing for Apheresis and Progenitor Cell Yield in Patients with Hematologic Malignancies and Solid Tumors"Tohoku J Exp Med.. 199. 111-118 (2003)

    • Description
      「研究成果報告書概要(欧文)」より

URL: 

Published: 2005-04-19  

Information User Guide FAQ News Terms of Use Attribution of KAKENHI

Powered by NII kakenhi