桑德霍夫病
克拉贝病
脂质运载蛋白
小胶质细胞
细胞外
生物
生物化学
前列腺素D2
神经节苷脂
酶
白质营养不良
前列腺素
细胞生物学
化学
内科学
疾病
免疫学
医学
炎症
作者
Ikuko Mohri,Masako Taniike,Issei Okazaki,Kuriko Kagitani‐Shimono,Kosuke Aritake,Takahisa Kanekiyo,Takashi Yagi,Shoichi Takikita,Hyungsuk Kim,Yoshihiro Urade,Kinuko Suzuki
标识
DOI:10.1111/j.1471-4159.2006.03753.x
摘要
Abstract Lipocalin‐type prostaglandin (PG) D synthase (L‐PGDS) is a dually functional protein, acting both as a PGD 2 ‐synthesizing enzyme and as an extracellular transporter of various lipophilic small molecules. L‐PGDS is expressed in oligodendrocytes (OLs) in the central nervous system and is up‐regulated in OLs of the twitcher mouse, a model of globoid cell leukodystrophy (Krabbe's disease). We investigated whether up‐regulation of L‐PGDS is either unique to Krabbe's disease or is a more generalized phenomenon in lysosomal storage disorders (LSDs), using LSD mouse models of Tay–Sachs disease, Sandhoff disease, GM 1 gangliosidosis and Niemann–Pick type C1 disease. Quantitative RT‐PCR revealed that L‐PGDS mRNA was up‐regulated in the brains of all these mouse models. In addition, strong L‐PGDS immunoreactivity was observed in OLs, but not in either astrocytes or microglia in these models. Thus, up‐regulation of L‐PGDS appears to be a common response of OLs in LSDs. Moreover, surface plasmon resonance analyses revealed that L‐PGDS binds GM 1 and GM 2 gangliosides, accumulated in neurons in the course of LSD, with high affinities ( K D = 65 and 210 n m , respectively). This suggests that L‐PGDS may play a role in scavenging harmful lipophilic substrates in LSD.
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