Deficient Biosynthesis of N - Acetylglucosaminyl - Phosphatidylinositol , the First hte ~ iiiediate of Glycosyl Phosphatidylinositol Anchor Biosynthesis , in Cell Lines Established from Patients with Paroxysmal Nocturnal Hemoglobinuria
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چکیده
Deficient Biosynthesis of N-AcetylglucosaminylPhosphatidylinositol, the First hte~iiiediate of Glycosyl Phosphatidylinositol Anchor Biosynthesis, in Cell Lines Established from Patients with Paroxysmal Nocturnal Hemoglobinuria By Minoru Takahashi,* Junji Takeda,* Shinichi Hirose,$ Robert Hyman, ll Norimitsu Inoue,* Toshio Miyata,* Etsuko Ueda,~S Teruo Kitani,~ M. Edward Medof,$ and Taroh Kinoshita*
منابع مشابه
Deficient Biosynthesis of N - Acetylglucosaminyl - Phosphatidylinositol , the First hte ~ iiiediate of Glycosyl Phosphatidylinositol Anchor Biosynthesis , in Cell Lines
Deficient Biosynthesis of N-AcetylglucosaminylPhosphatidylinositol, the First hte~iiiediate of Glycosyl Phosphatidylinositol Anchor Biosynthesis, in Cell Lines Established from Patients with Paroxysmal Nocturnal Hemoglobinuria By Minoru Takahashi,* Junji Takeda,* Shinichi Hirose,$ Robert Hyman, ll Norimitsu Inoue,* Toshio Miyata,* Etsuko Ueda,~S Teruo Kitani,~ M. Edward Medof,$ and Taroh Kinosh...
متن کاملDeficient biosynthesis of N-acetylglucosaminyl-phosphatidylinositol, the first intermediate of glycosyl phosphatidylinositol anchor biosynthesis, in cell lines established from patients with paroxysmal nocturnal hemoglobinuria
Paroxysmal nocturnal hemoglobinuria (PNH) is a hemolytic disorder caused by a deficiency of biosynthesis of the glycosyl phosphatidylinositol (GPI) anchor, but the biochemical defect is not completely understood. In the present study, we have analyzed affected cell lines established recently from two Japanese patients with PNH. Two lines of evidence indicate that these cells do not synthesize N...
متن کاملGlycosyl-phosphatidylinositol anchor synthesis in paroxysmal nocturnal hemoglobinuria: partial or complete defect in an early step.
The defect in the biosynthesis of the glycosyl-phosphatidyl inositol (GPI) anchor in paroxysmal nocturnal hemoglobinuria (PNH) appears to be in the initial steps. In biosynthetic studies using [3H]mannose, abnormal granulocytes of eight patients, and B lymphocytes transformed by Epstein-Barr virus of six different patients synthesized dolichyl phosphoryl mannose, but little or no later mannosyl...
متن کاملPhenotypic and functional characterization of a mouse model of targeted Pig-a deletion in hematopoietic cells.
BACKGROUND Somatic mutation in the X-linked phosphatidylinositol glycan class A gene (PIG-A) causes glycosyl phosphatidylinositol anchor deficiency in human patients with paroxysmal nocturnal hemoglobinuria. DESIGN AND METHODS We produced an animal model of paroxysmal nocturnal hemoglobinuria by conditional Pig-a gene inactivation (Pig-a(-/-)) in hematopoietic cells; mice carrying two lox sit...
متن کاملChromosomal assignment of genes involved in glycosylphosphatidylinositol anchor biosynthesis: implications for the pathogenesis of paroxysmal nocturnal hemoglobinuria.
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematologic disorder that affects both sexes equally. The biochemical defect in PNH resides in the incomplete enzymatic assembly of glycosylphosphatidylinositol (GPI) anchors used for surface protein attachment. In all PNH patients tested to date, the biosynthetic defect occurs at the addition of N-acetyl-glucosamine to the phospha...
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