クリバヤシ フトシ
Futoshi Kuribayashi
栗林 太 所属 川崎医科大学 医学部 基礎医学 生化学 職種 教授 |
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論文種別 | 原著 |
言語種別 | 英語 |
査読の有無 | 査読あり |
表題 | Poter D. Colin, Kuribayashi Futoshi, Parkar H.Mohamed, Roos Dirk, Kinnon Christine. |
掲載誌名 | 正式名:Biochemical journal 略 称:Biochem. J. |
巻・号・頁 | 315(2),571-575頁 |
著者・共著者 | Poter D. Colin, Kuribayashi Futoshi, Parkar H.Mohamed, Roos Dirk, Kinnon Christine. |
発行年月 | 1996/04 |
概要 | NADPH oxidase cytochrome b558 consists of two subunits, gp91-phox and p22-phox, defects of which result in chronic granulomatous disease (CGD). The nature of the interaction between these subunits has yet to be determined. Absence of p22-phox in autosomal CGD patient-derived B-cell lines results in detectable levels of an incompletely glycosylated gp91-phox precursor. We have detected this same precursor species in four cell lines from patients with the X-linked form of the disease due to mutations in gp91-phox. Such mutations should delineate regions of gp91-phox important for its biosynthesis, including stable association with p22-phox. One mutation mapped to the putative FAD-binding domain, one mapped to a potential haem-binding domain, and two involved the region encoded by exon 3. |