A5028984753- RNA regulation and disease
- Cytomegalovirus and herpesvirus research
- Genetic and Kidney Cyst Diseases
- Renal and related cancers
- Renal Diseases and Glomerulopathies
- Dialysis and Renal Disease Management
- Endoplasmic Reticulum Stress and Disease
- Metabolism and Genetic Disorders
- Biomedical Research and Pathophysiology
- Virus-based gene therapy research
- Amino Acid Enzymes and Metabolism
- Acute Kidney Injury Research
- Electrolyte and hormonal disorders
- RNA Research and Splicing
- Muscle and Compartmental Disorders
- Microtubule and mitosis dynamics
- Liver Disease Diagnosis and Treatment
- Heme Oxygenase-1 and Carbon Monoxide
- Ion Transport and Channel Regulation
- Amyloidosis: Diagnosis, Treatment, Outcomes
- Cardiovascular Function and Risk Factors
- Mitochondrial Function and Pathology
- Cerebrospinal fluid and hydrocephalus
- HIV Research and Treatment
- Protist diversity and phylogeny
University at Buffalo, State University of New York
2024
National Institute of Diabetes and Digestive and Kidney Diseases
2022-2024
National Institutes of Health
2022-2024
Tohoku University Hospital
2024
Georgetown University
2024
University of Florida
2024
The University of Tokyo
2014-2019
Memorial Hospital of South Bend
2011-2013
Mitsui Memorial Hospital
2011
Fibroblasts from patients with Tangier disease carrying ATP-binding cassette A1 (ABCA1) loss-of-function mutations are characterized by cardiolipin accumulation, a mitochondrial-specific phospholipid. Suppression of ABCA1 expression occurs in glomeruli diabetic kidney (DKD) and human podocytes exposed to DKD sera collected prior the development DKD. We demonstrated that siRNA knockdown led reduced oxygen consumption capabilities associated alterations oxidative phosphorylation (OXPHOS)...
Autosomal dominant polycystic kidney disease (ADPKD) constitutes the most inherited disease. Mutations in PKD1 and PKD2 genes, encoding polycystin 1 2 Ca2+ ion channels, respectively, result tubular epithelial cell-derived renal cysts. Recent clinical studies demonstrate oxidative stress to be present early ADPKD. Mitochondria comprise primary reactive oxygen species source also their main effector target; however, pathophysiological role of mitochondria ADPKD remains uncharacterized. To...
Tubulointerstitial fibrosis is a strong predictor of progression in patients with chronic kidney disease, and often accompanied by lipid accumulation renal tubules. However, the molecular mechanisms modulating relationship between lipotoxicity tubulointerstitial remain obscure. ATF6α, transcription factor unfolded protein response, reported to be an upstream regulator fatty acid metabolism. Owing their high energy demand, proximal tubular cells (PTCs) use acids as main source. We therefore...
Recent studies have reported intrinsic metabolic reprogramming in Pkd1 knock-out cells, implicating dysregulated cellular metabolism the pathogenesis of polycystic kidney disease. However, exact nature changes and their underlying cause remains controversial. We show herein that k o /ko renal epithelial cells impaired fatty acid utilization, abnormal mitochondrial morphology function, mitochondria kidneys ADPKD patients morphological alterations. further a C-terminal cleavage product...
Prolyl hydroxylase domain (PHD) inhibitors, which stimulate erythropoietin production through the activation of hypoxia-inducible factor (HIF), are novel therapeutic agents used for treating renal anemia. Several PHD including enarodustat, currently undergoing phase 2 or 3 clinical trials. Because HIF regulates a broad spectrum genes, inhibitors expected to have other effects in addition erythropoiesis, such as protection against metabolic disorders. However, whether beneficial would extend...
Abstract Sphingomyelin phosphodiesterase acid-like 3b (SMPDL3b) is a lipid raft enzyme that regulates plasma membrane (PM) fluidity. Here we report SMPDL3b excess, as observed in podocytes diabetic kidney disease (DKD), impairs insulin receptor isoform B-dependent pro-survival signaling by interfering with isoforms binding to caveolin-1 the PM. excess affects production of active sphingolipids resulting decreased ceramide-1-phosphate (C1P) content human vitro and cortexes db/db mice vivo....
Recent studies have highlighted the renoprotective effect of sirtuin1 (SIRT1), a deacetylase that contributes to cellular regulation. However, pathophysiologic role SIRT1 in podocytes remains unclear. Here, we investigated function podocytes. We first established podocyte-specific Sirt1 knockout (SIRT1(pod-/-)) mice. then induced glomerular disease by nephrotoxic serum injection. The increase urinary albumin excretion and BUN severity injury were all significantly greater SIRT1(pod-/-) mice...
Lipotoxicity plays an important role in the progression of chronic kidney damage via various mechanisms, such as endoplasmic reticulum stress. Several studies proposed renal lipotoxicity glomerular and tubular cells but effect lipid on erythropoietin (EPO)-producing (REP) interstitium has not been elucidated. Since anemia is caused by derangement EPO production REP cells, we evaluated palmitate, a representative long-chain saturated fatty acid, stress pathway. was suppressed palmitate...
A 44-year-old man with a 17-year history of Crohn's disease (CD) was referred to our nephrology department on suspicion drug-induced nephrotoxicity. Over the preceding 18 months, he had slowly progressive renal insufficiency slight urinary abnormalities. His activity been well controlled up that point 5-aminosalicylic acid and azathiopurine. Laboratory examination revealed proteinuria without hematuria an elevated serum creatinine level 1.4 mg/dl. Pathological amyloid (AA) deposition in...
PKD1 is the most commonly mutated gene causing autosomal dominant polycystic kidney disease (ADPKD). It encodes Polycystin-1 (PC1), a putative membrane protein that undergoes set of incompletely characterized post-transcriptional cleavage steps and has been reported to localize in multiple subcellular locations, including primary cilium mitochondria. However, direct visualization PC1 detailed characterization its binding partners remain challenging. We now report new mouse model with HA...
1型糖尿病の60歳男性．IgG4関連硬化性疾患による後腹膜線維症，同リンパ節腫脹，硬化性唾液腺炎のため経過観察していたが，血清総蛋白の上昇，腎機能障害と共に全身倦怠感，口渇が出現・増悪した．中枢性副腎不全，中枢性尿崩症を呈しており頭部MRIで下垂体炎を認めた．IgG4関連硬化性疾患による一連の病変でありステロイドが著効した．IgG4関連硬化性疾患による下垂体炎の貴重な症例を経験したので報告する．
Randomized trials have demonstrated that a phosphate binder ferric citrate (FeC) increases iron parameters in comparison with other binders, but the doses for FeC to improve stores safely not been clarified.We examined changes of and blood hemoglobin (Hb) 7 iron-deficient hemodialysis (HD) patients taking 750 mg/day as binder.The median serum transferrin saturation ferritin increased from 13% (interquartile range (IQR) 7-18) 28% (IQR 22-31; p = 0.010) 17 ng/ml 11-60) 106 58-176; 0.015) by 2...
HIV disease remains prevalent in the USA and chronic kidney a major cause of morbidity HIV-1-positive patients. Host double-stranded RNA (dsRNA)-activated protein kinase (PKR) is sensor for viral dsRNA, including HIV-1. We show that PKR inhibition by compound C16 ameliorates HIV-associated nephropathy (HIVAN) phenotype Tg26 transgenic mouse model, with reversal mitochondrial dysfunction. Combined analysis single-nucleus RNA-seq bulk data revealed oxidative phosphorylation was one most...
HIV disease remains prevalent in the USA and chronic kidney a major cause of morbidity HIV-1-positive patients. Host double-stranded RNA (dsRNA)-activated protein kinase (PKR) is sensor for viral dsRNA, including HIV-1. We show that PKR inhibition by compound C16 ameliorates HIV-associated nephropathy (HIVAN) phenotype Tg26 transgenic mouse model, with reversal mitochondrial dysfunction. Combined analysis single-nucleus RNA-seq bulk data revealed oxidative phosphorylation was one most...
HIV disease remains prevalent in the USA and chronic kidney a major cause of morbidity HIV-1-positive patients. Host double-stranded RNA (dsRNA)-activated protein kinase (PKR) is sensor for viral dsRNA, including HIV-1. We show that PKR inhibition by compound C16 ameliorates HIV-associated nephropathy (HIVAN) phenotype Tg26 transgenic mouse model, with reversal mitochondrial dysfunction. Combined analysis single-nucleus RNA-seq bulk data revealed oxidative phosphorylation was one most...
HIV disease remains prevalent in the USA and chronic kidney a major cause of morbidity HIV-1-positive patients. Host double-stranded RNA (dsRNA)-activated protein kinase (PKR) is sensor for viral dsRNA, including HIV-1. We show that PKR inhibition by compound C16 ameliorates HIV-associated nephropathy (HIVAN) phenotype Tg26 transgenic mouse model, with reversal mitochondrial dysfunction. Combined analysis single-nucleus RNA-seq bulk data revealed oxidative phosphorylation was one most...
Although hyponatremia and salt wasting are common in patients with HIV/AIDS, the understanding of their contributing factors is limited. HIV viral protein R (Vpr) contributes to HIV-associated nephropathy. To investigate effects Vpr on distal tubules expression level Slc12a3 gene, encoding sodium-chloride cotransporter (which responsible for sodium reabsorption nephron segments), single-nucleus RNA sequencing was performed kidney cortices from three wild-type (WT) transgenic (Vpr Tg) mice....