Two Siblings of Lewis Rats with a Congenital Homozygous Mutation ofGene Encoding the Large Subunit of Liver-Specific Microsomal TriglycerideTransfer Protein (MTP), Resulting in Pure Red Cell Aplasia and OneSibling with the Heterozygous Genetic Mutation of

スポンサーリンク

概要

• 論文の詳細を見る

• A homozygous sequence mutation of microsomal triglyceride transfer protein (MTP) was studied in three Lewis rat siblings. Two of the three siblings had a homozygous sequence mutation in the exon-1 of the large subunit gene of liver-specific MTP and one of the three siblings had a heterozygous mutation of the large subunit gene. The two rats with a homozygous mutation were confirmed to have pure red cell aplasia at the age of 130 days when they were sacrificed because of weakness. The findings of the two rats were as follows : 1) Body weights were decreased to 65% of normal rats. 2) Plasma apolipoprotein B (apoB) was 0.54-0.60g/L. 3) A homozygous sequence mutation of the large subunit gene of liver-specific MTP was confirmed by polymerase chain reaction (PCR). The specific products of genomic liver DNA were a small amount in the PCR. 4) Acanthocytes constituted 7-14% of the red cells in the peripheral blood (PB). 5) The myeloid to erythroid cell ratio was 10 : 1 in the bone marrow (BM). The BM tended to hypoplasia. 6) Peritubular damage and reduced erythropoietin (epo) secretion were found in the kidney. Amyloid degeneration of the tubules and perivasucular fibrosis were found in the male and the female sibling, respectively. 7) Thymus atrophy with a low % of CD8a+ cells was observed. The silent large subunit gene of genomic lymphocyte DNA was amplified actively in the PCR. 8) Iron was deposited in the small sized hepatocytes with a homozygous MTP gene mutation. In conclusion, silent MTP gene expression and tissue apoptotic changes developed with age in rats with a homozygous liver-specific MTP mutation.

• 新潟大学の論文

著者

• 中辻 理子

  Department Of Transfusion Hamamatsu University School Of Medicine

• Nakatsuji Tadako

  Department Of Transfusion Hamamatsu University School Of Medicine

• Tadako NAKATSUJI

  Department of Transfusion, Hamamatsu University School of Medicine

• 中辻 理子

  Department of Transfusion, Hamamatsu University School of Medicine

関連論文

• 長期生存高齢者急性白血病の1例
• 血漿蛋白異常のある患者の診断の進めかた (血液の病気--その理解と対策)
• Two Siblings of Lewis Rats with a Congenital Homozygous Mutation ofGene Encoding the Large Subunit of Liver-Specific Microsomal TriglycerideTransfer Protein (MTP), Resulting in Pure Red Cell Aplasia and OneSibling with the Heterozygous Genetic Mutation of
• Renal or Liver Apoptotic Changes Triggered by Inoculation ofHomogenic T Cells and Ensuring High IL-2R Expression on T Cell:A Comparison with Early Graft-versus-Host Reaction (GvHR)
• Effects of Splenectomy on Rat Polymorphonuclear NeutrophilAutoimmunity
• Iron-related Autoimmune Hemolysis: Inductive and Competi-tiveReactions Found in Lewis Rats
• Increased Hepatic Barr Body and Thymic Epithelial Cell Dysplasia Foundin Chronic Immune Responses to Rat Male (H-Y) Antigen
• 急性骨髄性白血病に移行したprimary acquired sideroblastic anemiaの1剖検例
• 多発性骨髄腫--Melphalan療法と予後因子
• 5年以上生存している急性白血病の3例
• 慢性骨髄性白血病の経過中に粟粒結核を合併した1例
• 寛解期に乳房巨大腫瘤形成をみた急性骨髄性白血病の1例
• 剣道踏み込み練習によるいわゆる行運血色素尿症の2例
• 白血病における肝障害
• T Cell Hypofunctions and Glomerular Sclerotic and Angiogenic Changes Found Both in Rats Received Unilateral Nephrectomy plus Transplantation of Syngeneic Mesenteric Lymph Nodes and in Rats Received Unilateral Nephrectomy plus Splenectomy
• One Lewis Rat with Homozygous Defect of the Serine Proteinase Inhibitor 2 (Spi-2) Gene and Two Lewis Rats with Heterozygous Spi-2 Gene Defect
• Alpha 1 Antitrypsin Defective Lewis Rats Injected with Heparin : Comparison of the Glomerular Changes with Those of Lewis Rats Produced Anti BSA Antibody
• Acceleration of thrombosis and hemolysis by fibrinolysis inhibiting factor and suppression by Ia antigen expressed on T cells in partially hepatectomized Lewis rats
• Better-Surviving Liver Grafts by the Injection of Anti-CD2 Antibody : The Important Roles of Host CD8^+ and CD2^+CD28^+ T Cells in Chronic Graft Rejection and β Type Platelet-Derived Growth Factor Receptor (PDGFR-β) Expression on Apoptotic Liver Grafts

もっと見る 閉じる

スポンサーリンク