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1991 Fiscal Year Final Research Report Summary

Study on Placental Sulfatase Deficiency ; Diagnosis, Treatment and Genetics

Research Project

Project/Area Number 01570940
Research Category

Grant-in-Aid for General Scientific Research (C)

Allocation TypeSingle-year Grants
Research Field Obstetrics and gynecology
Research InstitutionShowa University

Principal Investigator

YANAIHARA Takumi  Showa University School of Medicine, Professor, 医学部, 教授 (70102308)

Co-Investigator(Kenkyū-buntansha) HIRATO Kumiko  Showa University School of Medicine, Assistant, 医学部, 助手 (00102301)
Project Period (FY) 1989 – 1991
KeywordsPlacental sulfatase feficiency / Sulfatase / Placenta / Leukocytes / Ichthyosis / Estrogen excretion
Research Abstract

Placental sulfatase deficiency(PSD)is a rare disorder with low estrogen production due to placental enzymatic deficiency- PSD is diagnosed by in vitro placental enzyme assay. In Japan, we have confirmed 65 cases of this disorder since 1979. In the present investigation, we have analysed the clinical data of 65 cases including the mode of delivery, family pedigree, birth weight of the infants, placental weight, developent of ichthyosis during the period of 1-3 months after birth. In addition, sulfatasse activity in peripheral blood leukocytes isolated from normal women and men, and the activity was compared to that from the PSD mothers and the infants. Deficiency of the sulfatase of the placenta obtained was confirmed by in vitro incubation with ^<14>C-DHA-Sulfate and the activity was expressed as the amounts of the free steroid. Characteristic absence and or an extremely low level of sulfatase activity was noticed in all the placenta obtained. In regard to the mode of delivery, pregnan … More cy was terminated by cesarean section in 47% of the cases. Spontaneous labor was observed only 27%. Sixty infants developed ichythyosis and all affected fetuses were males. The steroid sulfatase activity in female leukocytes was significantly stronger than that in normal males. Sulfatase in leukocytes obtained from the PSD babies and recessive X-linked ichthyosis patients had lower sensitivity. In the case of the mother affected with PSD, the activity was less than the half of that in normal men and the levels did not overlap with that in normal women suggesting that the measurement of leukocyte sulfatase activity would be a clinically useful tool for the diagnosis of PS @-arriers and pedigree analysis.
Steroid sulfatase was purified approximately 170-fold from normal human placental microsome and properties of the enzyme were, Nnvestigated. The purified sulfatase showed a molecular weight of 500 -, OKDA on HPLC gel filtration, whereas the enzyme migrated as a molecular mass of 73KDa on sodium dodecyl sulfate polyacrylamide gel electro phoresis. Amino acid analysis revealed that the purified human placental sulfatase did not contain cystein residue. Addition of phosphatidylcholine did not enhance the enzyme activity in the placental microsomes obtained from the patients with PSD after solvilization and chromatofocusing. This result indicates that PSD is the result of defect of the enzyme rather than the defect in membrane-enzyme structure. Less

  • Research Products

    (8 results)

All Other

All Publications (8 results)

  • [Publications] KUMIKO HIRATO AND TAKUMI YANAIHARA: "Serum Steroid Hormone Levels in Neonates Born from the Mother with Placental Sulfatase Deficiency" Endocrinol.Japon.37. 731-739 (1990)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] 鈴木 隆,平戸 久美子,范姜 正仁,斉藤 裕,矢内原 巧,真井 康博,真井 徳幸: "胎盤性Sulfatase欠損症の双胎例" 日産婦関東連合地方部会報. 53. 5-8 (1991)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] KUMIKO HIRATO,TAKASHI SUZUKI,TAKAAKI HONDO,HIROSHI SAITO,TAKUMI YANAIHARA: "Steroid Sulfatase Activities in Human Leukocytes:Biochemical and Clinical Aspects" Endocrinol.Japon.38. 597-602 (1991)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] TAKASHI SUZUKI,KUMIKO HIRATO,TAKUMI YANAIHARA,TSUYOKI KADOFUKU,TSUNEO SATO,MINORU HASHINO,NOBORU YANAIHARA: "Purification and properties of steroid sulfatase from human placenta" Endocrinol.Japon.39. (1992)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Kumiko Hirato, et al.: "Serum steroid hormone levels in neonates born from the mother with placental sulfatase deficiency" Endocrinol Jpn. 37 : 5. 739-747 (1990)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Takashi Suzuki, et al.: "A case of placental sulfatase deficiency with twin pregnancy" Acta Obstet Gynecol Jap of Kanto Area.53. 5-8 (1991)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Kumiko Hirato, et al.: "Steroid sulfatase activities in human leukocytes : Biochemical and clinical aspects" Endocrinol Jpn. 38 : 6. 597-602 (1991)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Takashi Suzuki, et al.: "Purification and properties of steroid sulfatase from human placenta" Endocrinol Jpn.

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

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Published: 1993-03-16  

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