Abstract：
:Niemann-Pick disease type C (NPC) is a recessive hereditary disease caused by mutation of the NPC1 or NPC2 gene. It is characterized by abnormality of cellular cholesterol trafficking with severe neuronal and hepatic injury. In this study, we investigated the potential of glycoprotein nonmetastatic melanoma protein B (GPNMB) to act as a biomarker reflecting the therapeutic effect of 2-hydroxypropyl-β-cyclodextrin (HP-β-CD) in an NPC mouse model. We measured serum, brain, and liver expression levels of GPNMB, and evaluated their therapeutic effects on NPC manifestations in the brain and liver after the intracerebroventricular administration of HP-β-CD in Npc1 gene-deficient (Npc1-/-) mice. Intracerebroventricular HP-β-CD inhibited cerebellar Purkinje cell damage in Npc1-/- mice and significantly reduced serum and cerebellar GPNMB levels. Interestingly, we also observed that the intracerebral administration significantly reduced hepatic GPNMB expression and elevated serum ALT in Npc1-/- mice. Repeated doses of intracerebroventricular HP-β-CD (30 mg/kg, started at 4 weeks of age and repeated every 2 weeks) drastically extended the lifespan of Npc1-/- mice compared with saline treatment. In summary, our results suggest that GPNMB level in serum is a potential biomarker for evaluating the attenuation of NPC pathophysiology by intracerebroventricular HP-β-CD treatment.
journal_name
Int J Mol Scijournal_title
International journal of molecular sciencesauthors
Fukaura M,Ishitsuka Y,Shirakawa S,Ushihama N,Yamada Y,Kondo Y,Takeo T,Nakagata N,Motoyama K,Higashi T,Arima H,Kurauchi Y,Seki T,Katsuki H,Higaki K,Matsuo M,Irie Tdoi
10.3390/ijms22010452subject
Has Abstractpub_date
2021-01-05 00:00:00issue
1issn
1422-0067pii
ijms22010452journal_volume
22pub_type
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