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2017 Highlights


August 01, 2017

Volume 23, Issue 8

Inflammation-dependent cerebrospinal fluid hypersecretion by the choroid plexus epithelium in posthemorrhagic hydrocephalus

The choroid plexus epithelium (CPE) secretes higher volumes of fluid (cerebrospinal fluid, CSF) than any other epithelium and simultaneously functions as the blood-CSF barrier to gate immune cell entry into the central nervous system. Posthemorrhagic hydrocephalus (PHH), an expansion of the cerebral ventricles due to CSF accumulation following intraventricular hemorrhage (IVH), is a common disease usually treated by...


February 07, 2017

Volume 25, Issue 2

WNK Kinase Signaling in Ion Homeostasis and Human Disease

WNK kinases, along with their upstream regulators (CUL3/KLHL3) and downstream targets (the SPAK/OSR1 kinases and the cation-Cl- cotransporters [CCCs]), comprise a signaling cascade essential for ion homeostasis in the kidney and nervous system. Recent work has furthered our understanding of the WNKs in epithelial transport, cell volume homeostasis, and GABA signaling, and uncovered novel roles for this pathway in immune cell function and cell proliferation.

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December 01, 2017

Volume 74, Issue 12

Xp22.2 Chromosomal Duplication in Familial Intracranial Arachnoid Cyst

Arachnoid cysts (ACs) are congenital fluid-filled malformations that account for approximately 1% of all intracranial, space-occupying lesions in the central nervous system. Despite an estimated prevalence of 1.4%, little is known about the pathogenesis of these presumed developmental anomalies of the arachnoid. The coincidence of ACs in known mendelian cystic disorders, such as autosomal dominant polycystic kidney disease, along with rare clinical reports of familial AC occurrence, suggests a genetic basis for the disorder. However, no gene or chromosomal abnormalities have been detected in...

Heubl, M., Zhang, J., Pressey, J. C., Al Awabdh, S., Renner, M., Gomez-Castro, F., … Lévi, S. (2017). GABAA receptor dependent synaptic inhibition rapidly tunes KCC2 activity via the Cl−-sensitive WNK1 kinase. Nature Communications, 8(1), 1776.
Furey, C. G., Timberlake, A. T., Nelson-Williams, C., Duran, D., Li, P., Jackson, E. M., & Kahle, K. T. (2017). Xp22.2 Chromosomal Duplication in Familial Intracranial Arachnoid Cyst. JAMA Neurology, 74(12), 1503.
Timberlake, A. T., Furey, C. G., Choi, J., Nelson-Williams, C., Loring, E., Galm, A., … Lifton, R. P. (2017). De novo mutations in inhibitors of Wnt, BMP, and Ras/ERK signaling pathways in non-syndromic midline craniosynostosis. Proceedings of the National Academy of Sciences, 114(35), E7341–E7347.
Zhao, H., Nepomuceno, R., Gao, X., Foley, L. M., Wang, S., Begum, G., … Sun, D. (2017). Deletion of the WNK3-SPAK kinase complex in mice improves radiographic and clinical outcomes in malignant cerebral edema after ischemic stroke. Journal of Cerebral Blood Flow & Metabolism, 37(2), 550–563.
Duran, D., Messina, R. D., Beslow, L. A., Montejo, J. D., Karimy, J. K., Gavankar Furey, C., … Kahle, K. T. (2017). Malignant Cerebellar Edema Subsequent to Accidental Prescription Opioid Intoxication in Children. Frontiers in Neurology, 8, 362.
Karimy, J. K., Zhang, J., Kurland, D. B., Theriault, B. C., Duran, D., Stokum, J. A., … Kahle, K. T. (2017). Inflammation-dependent cerebrospinal fluid hypersecretion by the choroid plexus epithelium in posthemorrhagic hydrocephalus. Nature Medicine, 23(8), 997–1003.
Zhang, J., Karimy, J. K., Delpire, E., & Kahle, K. T. (2017). Pharmacological targeting of SPAK kinase in disorders of impaired epithelial transport. Expert Opinion on Therapeutic Targets, 21(8), 795–804.
Vasquez, J. C., Huttner, A., Zhang, L., Marks, A., Chan, A., Baehring, J. M., … Dhodapkar, K. M. (2017). SOX2 immunity and tissue resident memory in children and young adults with glioma. Journal of Neuro-Oncology, 134(1), 41–53.
Glykys, J., Dzhala, V., Egawa, K., Kahle, K. T., Delpire, E., & Staley, K. (2017). Chloride Dysregulation, Seizures, and Cerebral Edema: A Relationship with Therapeutic Potential. Trends in Neurosciences, 40(5), 276–294.
Gaillard, J., Klein, J., Duran, D., Storey, A., Scott, R. M., Kahle, K., & Smith, E. R. (2017). Incidence, clinical features, and treatment of familial moyamoya in pediatric patients: a single-institution series. Journal of Neurosurgery: Pediatrics, 19(5), 553–559.
Shekarabi, M., Zhang, J., Khanna, A. R., Ellison, D. H., Delpire, E., & Kahle, K. T. (2017). WNK Kinase Signaling in Ion Homeostasis and Human Disease. Cell Metabolism, 25(2), 285–299.
Duran, D., Hadzipasic, M., & Kahle, K. T. (2017). Mystery Case: Acute hydrocephalus caused by radiographically occult fourth ventricular outlet obstruction. Neurology, 88(5), e36–e37.
Delpire, E., & Kahle, K. T. (2017). The KCC3 cotransporter as a therapeutic target for peripheral neuropathy. Expert Opinion on Therapeutic Targets, 21(2), 113–116.
Grunwald, Z., Beslow, L. A., Urday, S., Vashkevich, A., Ayres, A., Greenberg, S. M., … Sheth, K. N. (2017). Perihematomal Edema Expansion Rates and Patient Outcomes in Deep and Lobar Intracerebral Hemorrhage. Neurocritical Care, 26(2), 205–212.
Bhuiyan, M. I. H., Song, S., Yuan, H., Begum, G., Kofler, J., Kahle, K. T., … Sun, D. (2017). WNK-Cab39-NKCC1 signaling increases the susceptibility to ischemic brain damage in hypertensive rats. Journal of Cerebral Blood Flow & Metabolism, 37(8), 2780–2794.
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