Hervé Husson

Circulating Levels of Inorganic Pyrophosphate Influence the Risk and Clinical Outcomes in Calciphylaxis

Sagar U. Nigwekar, Api Chewcharat, Kevin O’Brien, Enock Arthur et autres

C alciphylaxis is a rare, life-limiting syndrome defined by vascular calcification and neointimal proliferation. 1 This pathological process induces microvascular occlusions within the subcutaneous and dermal adipose tissue, resulting in intensely painful, ischemic skin lesions.Primarily affecting patients with end-stage kidney disease (ESKD), …

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Therapeutic approaches for the treatment of genetic and acquired cardiovascular calcification

Kevin D. O’Brien, Hervé Husson, Yves Sabbagh

Vascular calcification, the deposition of calcium-phosphate crystals in the vasculature, occurs through a complex interplay between cellular processes and biochemical factors that are yet to be entirely defined. Vascular calcification results in stiffening of the arteries and ultimately cardiovascular complications. Deposition can …

Recombinant ectonucleotide pyrophosphatase/phosphodiesterase 1 (ENPP1) decreases vascular calcification and prevents osteomalacia in a rat model of chronic kidney disease

Kevin D. O’Brien, Lisa Laurion, Caitlin Sullivan, Jennifer Howe et autres

Chronic kidney disease (CKD) impacts a large percentage of the global population. Chronic kidney disease-mineral bone disorder (MBD) is the broad term describing alterations in key circulating factors involved in mineralization, ectopic calcification, and bone abnormalities. Cardiovascular complications, involving vascular calcification are …

Editorial Expression of Concern: RAFTK/PYK2-dependent and -independent apoptosis in multiple myeloma cells

Dharminder Chauhan, Teru Hideshima, Pramod S. Pandey, Steve P. Treon et autres

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Glucosylceramide synthase modulation ameliorates murine renal pathologies and promotes macrophage effector function in vitro

Agnes Cheong, Florin L. Craciun, Hervé Husson, JOSEPH H. GANS et autres

While significant advances have been made in understanding renal pathophysiology, less is known about the role of glycosphingolipid (GSL) metabolism in driving organ dysfunction. Here, we used a small molecule inhibitor of glucosylceramide synthase to modulate GSL levels in three mouse models …

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Glucosylceramide synthase inhibition protects against cardiac hypertrophy in chronic kidney disease

Gabriel C. Baccam, Jian Xie, Xin Jin, Hye Jung Park et autres

A significant population of patients with chronic kidney disease (CKD) develops cardiac hypertrophy, which can lead to heart failure and sudden cardiac death. Soluble klotho (sKL), the shed ectodomain of the transmembrane protein klotho, protects the heart against hypertrophic growth. We have …

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Anti-microRNA-21 Therapy on Top of ACE Inhibition Delays Renal Failure in Alport Syndrome Mouse Models

Diana M. Rubel, Joseph Boulanger, Florin L. Craciun, Ethan Xu et autres

Col4a3−/− Alport mice serve as an animal model for renal fibrosis. MicroRNA-21 (miR-21) expression has been shown to be increased in the kidneys of Alport syndrome patients. Here, we investigated the nephroprotective effects of Lademirsen anti-miR-21 therapy. We used a fast-progressing Col4a3−/− …

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Correction of cilia structure and function alleviates multi-organ pathology in Bardet–Biedl syndrome mice

Hervé Husson, Nikolay O. Bukanov, Sarah E. Moreno, Mandy M. Smith et autres

Bardet-Biedl syndrome (BBS) is a pleiotropic autosomal recessive ciliopathy affecting multiple organs. The development of potential disease-modifying therapy for BBS will require concurrent targeting of multi-systemic manifestations. Here, we show for the first time that monosialodihexosylganglioside accumulates in Bbs2-/- cilia, indicating impairment …

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Differences in the timing and magnitude ofPkd1gene deletion determine the severity of polycystic kidney disease in an orthologous mouse model of ADPKD

Kelly A. Rogers, Sarah E. Moreno, Laurie A. Smith, Hervé Husson et autres

Development of a disease-modifying therapy to treat autosomal dominant polycystic kidney disease (ADPKD) requires well-characterized preclinical models that accurately reflect the pathology and biochemical changes associated with the disease. Using a Pkd1 conditional knockout mouse, we demonstrate that subtly altering the timing …

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CaMKII as a pathological mediator of ER stress, oxidative stress, and mitochondrial dysfunction in a murine model of nephronophthisis

Christina Bracken, Philippe Beauverger, Olivier Duclos, Ryan J. Russo et autres

Polycystic kidney diseases (PKDs) are genetic diseases characterized by renal cyst formation with increased cell proliferation, apoptosis, and transition to a secretory phenotype at the expense of terminal differentiation. Despite recent progress in understanding PKD pathogenesis and the emergence of potential therapies, …

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Reduction of ciliary length through pharmacologic or genetic inhibition of CDK5 attenuates polycystic kidney disease in a model of nephronophthisis

Hervé Husson, Sarah E. Moreno, Laurie A. Smith, Mandy M. Smith et autres

Polycystic kidney diseases (PKDs) comprise a subgroup of ciliopathies characterized by the formation of fluid-filled kidney cysts and progression to end-stage renal disease. A mechanistic understanding of cystogenesis is crucial for the development of viable therapeutic options. Here, we identify CDK5, a …

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Exome Capture Reveals ZNF423 and CEP164 Mutations, Linking Renal Ciliopathies to DNA Damage Response Signaling

Moumita Chaki, Rannar Airik, Amiya Kumar Ghosh, Rachel H. Giles et autres

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