[HTML][HTML] A sensitive and specific LC-MS/MS method for rapid diagnosis of Niemann-Pick C1 disease from human plasma [S]

…, AO Speak, DT Te Vruchte, FM Platt, H Fujiwara… - Journal of lipid …, 2011 - ASBMB
Niemann-Pick type C1 (NPC1) disease is a rare, progressively fatal neurodegenerative
disease for which there are no FDA-approved therapies. A major barrier to developing new …

Modulation of cellular S1P levels with a novel, potent and specific inhibitor of sphingosine kinase-1

…, JA Scholten, J Wendling, H Fujiwara… - Biochemical …, 2012 - portlandpress.com
SphK (sphingosine kinase) is the major source of the bioactive lipid and GPCR (G-protein-coupled
receptor) agonist S1P (sphingosine 1-phosphate). S1P promotes cell growth, survival …

Overexpression of 20-oxidase confers a gibberellin-overproduction phenotype in Arabidopsis

S Huang, AS Raman, JE Ream, H Fujiwara… - Plant …, 1998 - academic.oup.com
In the gibberellin (GA) biosynthesis pathway, 20-oxidase catalyzes the oxidation and
elimination of carbon-20 to give rise to C 19 -GAs. All bioactive GAs are C 19 -GAs. We have …

A new glucocerebrosidase chaperone reduces α-synuclein and glycolipid levels in iPSC-derived dopaminergic neurons from patients with Gaucher disease and …

…, W Zheng, P Sullivan, H Fujiwara… - Journal of …, 2016 - Soc Neuroscience
Among the known genetic risk factors for Parkinson disease, mutations in GBA1, the gene
responsible for the lysosomal disorder Gaucher disease, are the most common. This genetic …

[HTML][HTML] Early-onset metabolic syndrome in mice lacking the intestinal uric acid transporter SLC2A9

BJ DeBosch, O Kluth, H Fujiwara, A Schürmann… - Nature …, 2014 - nature.com
Excess circulating uric acid, a product of hepatic glycolysis and purine metabolism, often
accompanies metabolic syndrome. However, whether hyperuricaemia contributes to the …

T-cell glucocorticoid receptor is required to suppress COX-2-mediated lethal immune activation

JA Brewer, B Khor, SK Vogt, LM Muglia, H Fujiwara… - Nature medicine, 2003 - nature.com
Glucocorticoids, acting through the glucocorticoid receptor, potently modulate immune
function and are a mainstay of therapy for treatment of inflammatory conditions, autoimmune …

[HTML][HTML] Palmitate and lipopolysaccharide trigger synergistic ceramide production in primary macrophages

…, HM Machkovech, L He, R Sidhu, H Fujiwara… - Journal of Biological …, 2013 - ASBMB
Macrophages play a key role in host defense and in tissue repair after injury. Emerging
evidence suggests that macrophage dysfunction in states of lipid excess can contribute to the …

Targeting the mitochondrial pyruvate carrier attenuates fibrosis in a mouse model of nonalcoholic steatohepatitis

…, I Nalbantoglu, WG McDonald, C Holley, H Fujiwara… - Hepatology, 2017 - journals.lww.com
Diseases of the liver related to metabolic syndrome have emerged as the most common and
undertreated hepatic ailments. The cause of nonalcoholic fatty liver disease is the aberrant …

A murine Niemann-Pick C1 I1061T knock-in model recapitulates the pathological features of the most prevalent human disease allele

…, B Tortelli, J Zhang, H Fujiwara… - Journal of …, 2015 - Soc Neuroscience
Niemann-Pick Type C1 (NPC1) disease is a rare neurovisceral, cholesterol–sphingolipid
lysosomal storage disorder characterized by ataxia, motor impairment, progressive intellectual …

[HTML][HTML] Identification of Niemann-Pick C1 disease biomarkers through sphingolipid profiling

M Fan, R Sidhu, H Fujiwara, B Tortelli, J Zhang… - Journal of lipid …, 2013 - ASBMB
Niemann-Pick type C (NPC)1 is a rare neurodegenerative disease for which treatment
options are limited. A major barrier to development of effective treatments has been the lack of …