• Title/Summary/Keyword: Hermansky-Pudlak syndrome

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Antioxidant Activities in the Lung of Murine Hermansky-Pudlak Syndrome (HPS) Model: Effect of Ionizing Radiation (Hermansky-Pudlak Syndrome (HPS) 생쥐 모델의 폐 항산화계 활성: 방사선의 영향)

  • Shin, Ho-Sang;Yang, Woo-Jung;Choi, Eun-Mi
    • Environmental Analysis Health and Toxicology
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    • v.22 no.1 s.56
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    • pp.9-17
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    • 2007
  • Hermansky-Pudlak Syndrome (HPS) 환자에서 조기에 발생되는 폐섬유화의 원인을 알아보고자, 생쥐 HPS 모델인 ep/ep,pe/pe 돌연변이종의 폐 항산화계의 환성과 방사선에 대한 반응을 측정하였다. HPS 폐에서는 대조군에 비해 glutathione이 더 산화되어 있었고, catalase, glutathione S-transferase(GST) 등의 항산화효소의 활성이 저하되어 있었으며, 10 Gy의 방사선을 조사하였을 때, glutathione 양이 감소하였고, 대조군 폐에서 보여지는 방사선에 의한 ${\gamma}$-glutamylcysteine ligase(GCL), glutathione peroxidase(GPx) 활성의 유의성 있는 증가가 관찰되지 않았다. 이 결과로부터 HPS 환자의 폐는 항산화계 활성이 저하되어 있을 뿐 아니라, 산화적 스트레스가 가해 졌을 때 적응 반응이 매우 취약하여 산화적 환경에 노출된 폐의 병증을 유발할 수 있음을 추측할 수 있다.

Genetic Quality Control of the Rat Strains at the National Bio Resource Project-Rat

  • Kuramoto, Takashi;Nakanishi, Satoshi;Yamasaki, Ken-ichi;Kumafuji, Kenta;Sakakibara, Yuichi;Neoda, Yuki;Takizawa, Akiko;Kaneko, Takehito;Otsuki, Mito;Hashimoto, Ryoko;Voigt, Birger;Mashimo, Tomoji;Serikawa, Tadao
    • Interdisciplinary Bio Central
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    • v.2 no.4
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    • pp.12.1-12.7
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    • 2010
  • The National Bio Resource Project-Rat (NBRP-Rat) comprises the largest bank of laboratory rat (Rattus norvegicus) strains in the world. Its main focus is to develop infrastructure that will facilitate the systematic collection, preservation, and provision of rat strains. To breed effectively more than 180 rat strains in living stock, we establish the genetic control system in which a systematic set of genetic diagnoses and genetic monitoring are included. Genetic monitoring is performed by using 20 polymorphic markers. Monitoring is carried out when a living animal stock is re-established by using cryopreserved embryos or sperm or when a rat strain is first introduced to the NBRP-Rat by a depositor. Additional monitoring is then carried out on each strain every two years. Genetic diagnosis is performed largely by employing the Amp-FTA method. Protocols which detail how to perform a genetic diagnosis of 11 transgenes and 24 mutations have been made. Among the mutations, nine can be detected by simple gel electrophoresis of the PCR products, 11 by restriction enzyme treatment of the PCR products, and four by direct PCR product sequencing. Using this genetic control system, the NBRP-Rat can guarantee the genetic quality of its rat strains.