Lipocalin-type prostaglandin D synthase is up-regulated in oligodendrocytes in lysosomal storage diseases and binds gangliosides

Ikuko Mohri, Masako Taniike, Issei Okazaki, Kuriko Kagitani-Shimono, Kosuke Aritake, Takahisa Kanekiyo, Takashi Yagi, Shoichi Takikita, Hyung Suk Kim, Yoshihiro Urade, Kinuko Suzuki

Research output: Contribution to journalArticlepeer-review

45 Scopus citations


Lipocalin-type prostaglandin (PG) D synthase (L-PGDS) is a dually functional protein, acting both as a PGD2-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, GM1 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 GM1 and GM2 gangliosides, accumulated in neurons in the course of LSD, with high affinities (KD = 65 and 210 nm, respectively). This suggests that L-PGDS may play a role in scavenging harmful lipophilic substrates in LSD.

Original languageEnglish (US)
Pages (from-to)641-651
Number of pages11
JournalJournal of neurochemistry
Issue number3
StatePublished - May 2006


  • GM gangliosidosis
  • Niemann-Pick type C1 disease
  • Oligodendrocytes
  • Prostaglandin D synthase
  • Sandhoff disease
  • Tay-Sachs disease

ASJC Scopus subject areas

  • Biochemistry
  • Cellular and Molecular Neuroscience


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