Is this you? As a journalist, you can create a free Muck Rack account to customize your profile, list your contact preferences, and upload a portfolio of your best work.
Claim your profile
Articles by Sharan Paul
A human Staufen1 BAC transgenic mouse exhibits abnormal autophagy and neurodegeneration across the central nervous system
Abstract RNA-binding proteins (RBPs) play an essential role in development, normal functioning, and human disease. Staufen1 (STAU1) is an RBP that regulates mRNA degradation and subcellular localization, and is part of the ATXN2 protein complex.
Viral vector-mediated SLC9A6 gene replacement reduces cerebellar dysfunction in the shaker rat model of Christianson syndrome
Abstract Christianson syndrome (CS) is an x-linked recessive neurodevelopmental and neurodegenerative condition characterized by severe intellectual disability, cerebellar degeneration, ataxia, and epilepsy. Mutations to the SLC9A6 gene are responsible for CS, and we recently demonstrated that a mutation to the rat Slc9a6 gene causes a similar phenotype in the spontaneous shaker rat model, which exhibits cerebellar degeneration with motor dysfunction.
GGC expansion in ZFHX3 causes SCA4 and impairs autophagy
The authors have declared no competing interest. This work was supported by NIH grant R35127253 to SMP. SO received funding from the German Research Foundation DFG (DFG OS 647/1-1). NGS sequencing methods were performed with the support of the DFG-funded NGS Competence Center Tuebingen (INST 37/1049-1). JP was supported by the Clinician Scientist program PRECISE.net funded by the Else Kroener-Fresenius-Stiftung.
Cholecystokinin 1 receptor activation restores normal mTORC1 signaling and is protective to Purkinje cells of SCA mice
Highlights • In murine Purkinje neurons expressing ATXN1[82Q], mTORC1 signaling is depressed • In murine Purkinje neurons expressing ATXN2[127Q], mTORC1 signaling is enhanced • CCK1R activation restores Purkinje cell function/morphology and mTORC1 signaling Summary Spinocerebellar ataxias (SCAs) are a group of genetic diseases characterized by progressive ataxia and neurodegeneration, often in cerebellar Purkinje neurons.
Splicing Control of Pontocerebellar Development
Sharan Paul Sharan Paul Correspondence Corresponding author Affiliations Department of Neurology, University of Utah, 175 North Medical Drive East, 5th Floor, Salt Lake City, UT 84132, USA Search for articles by this author Daniel R. Scoles Daniel R. Scoles Correspondence Corresponding author Affiliations Department of Neurology, University of Utah, 175 North Medical Drive East, 5th Floor, Salt Lake City, UT 84132, USA Search for articles by this author Stefan M. Pulst Stefan M.
Staufen 1 amplifies pro-apoptotic activation of the unfolded protein response
New Results View ORCID ProfileMandi Gandelman, Warunee Dansithong, Karla P Figueroa, Sharan Paul, Daniel R Scoles, Stefan M Pulst doi: https://doi.org/10.1101/820225 Abstract Staufen-1 (STAU1) is an RNA binding protein that becomes highly overabundant in numerous neurodegenerative disease models, including those carrying mutations in presenilin1 (PSEN1), microtubule associated protein tau (MAPT), huntingtin (HTT), TAR DNA-binding protein-43 gene (TARDBP) or C9orf72.
Staufen1 links RNA stress granules and autophagy in a model of neurodegeneration
An association of STAU1 and SGs in brain oligodendrocytes and other cultured cells was previously described24,25. Because ATXN2 is a component of SGs16,17, we investigated if ATXN2 and STAU1 co-localized under conditions of stress. The specificity of anti-Staufen antibody was confirmed by multiple methods (Supplementary Fig. 1a–d). Following exposure of HEK-293 cells to arsenite (oxidative stress), we assessed ATXN2 and STAU1 co-localization by immunofluorescence.
Show More
loading
Actions
Is this you?
As a journalist, you can create a free Muck Rack account to customize your profile, list your contact preferences, and upload a portfolio of your best work.Get in touch with Sharan
Contact Sharan, search articles and posts on X, monitor coverage, and track replies from one place.
Learn more about Muck Rack