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Two Siblings of Lewis Rats with a Congenital Homozygous Mutation of Gene Encoding the Large Subunit of Liver-Specific Microsomal Triglyceride Transfer Protein (MTP), Resulting in Pure Red Cell Aplasia and One Sibling with the Heterozygous Genetic Mutation of the Liver MTP Gene
http://hdl.handle.net/10191/1934
http://hdl.handle.net/10191/19348e505ca7-99c6-4c9a-a4ae-6dd4ccd514f1
名前 / ファイル | ライセンス | アクション |
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KJ00000007273.pdf (2.6 MB)
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Item type | 紀要論文 / Departmental Bulletin Paper(1) | |||||
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公開日 | 2007-05-10 | |||||
タイトル | ||||||
タイトル | Two Siblings of Lewis Rats with a Congenital Homozygous Mutation of Gene Encoding the Large Subunit of Liver-Specific Microsomal Triglyceride Transfer Protein (MTP), Resulting in Pure Red Cell Aplasia and One Sibling with the Heterozygous Genetic Mutation of the Liver MTP Gene | |||||
タイトル | ||||||
言語 | en | |||||
タイトル | Two Siblings of Lewis Rats with a Congenital Homozygous Mutation of Gene Encoding the Large Subunit of Liver-Specific Microsomal Triglyceride Transfer Protein (MTP), Resulting in Pure Red Cell Aplasia and One Sibling with the Heterozygous Genetic Mutation of the Liver MTP Gene | |||||
言語 | ||||||
言語 | eng | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | rat | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | apolipoprotein B | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | microsomal triglyceride transfer protein | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | acanthocyte | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | pure red cell aplasia | |||||
資源タイプ | ||||||
資源 | http://purl.org/coar/resource_type/c_6501 | |||||
タイプ | departmental bulletin paper | |||||
著者 |
NAKATSUJI, Tadako
× NAKATSUJI, Tadako |
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抄録 | ||||||
内容記述タイプ | Abstract | |||||
内容記述 | 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. | |||||
書誌情報 |
Acta medica et biologica en : Acta medica et biologica 巻 48, 号 4, p. 127-133, 発行日 2000-12 |
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出版者 | ||||||
出版者 | Niigata University School of Medicine | |||||
ISSN | ||||||
収録物識別子タイプ | ISSN | |||||
収録物識別子 | 05677734 | |||||
書誌レコードID | ||||||
収録物識別子タイプ | NCID | |||||
収録物識別子 | AA00508361 | |||||
著者版フラグ | ||||||
値 | publisher |