Výsledky vyhledávání - "H Jürgen, Wenzel"

Akademický článek

Astroglial-targeted expression of the fragile X CGG repeat premutation in mice yields RAN translation, motor deficits and possible evidence for cell-to-cell propagation of FXTAS pathology

Autor: H. Jürgen Wenzel, Karl D. Murray, Saif N. Haify, Michael R. Hunsaker, Jared J. Schwartzer, Kyoungmi Kim, Albert R. La Spada, Bryce L. Sopher, Paul J. Hagerman, Christopher Raske, Lies-Anne W.F.M. Severijnen, Rob Willemsen, Renate K. Hukema, Robert F. Berman

Publikováno v: Acta Neuropathologica Communications, Vol 7, Iss 1, Pp 1-22 (2019)

Abstract The fragile X premutation is a CGG trinucleotide repeat expansion between 55 and 200 repeats in the 5′-untranslated region of the fragile X mental retardation 1 (FMR1) gene. Human carriers of the premutation allele are at risk of developin

Externí odkaz: https://doaj.org/article/4134f0078ae64270bd17af8e8378a6cd

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Astroglial-targeted expression of the fragile X CGG repeat premutation in mice yields RAN translation, motor deficits and possible evidence for cell-to-cell propagation of FXTAS pathology

Autor: Kyoungmi Kim, Michael R. Hunsaker, Lies Anne Severijnen, Saif N Haify, Rob Willemsen, Albert R. La Spada, H. Jürgen Wenzel, Paul J. Hagerman, Christopher Raske, Bryce L. Sopher, Robert F. Berman, Karl D Murray, Renate K. Hukema, Jared J. Schwartzer

Publikováno v: Acta Neuropathologica Communications
Acta Neuropathologica Communications, Vol 7, Iss 1, Pp 1-22 (2019)
Acta neuropathologica communications, vol 7, iss 1
Acta neuropathologica communications (online), 7:27. BioMed Central Ltd.

The fragile X premutation is a CGG trinucleotide repeat expansion between 55 and 200 repeats in the 5′-untranslated region of the fragile X mental retardation 1 (FMR1) gene. Human carriers of the premutation allele are at risk of developing the lat

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b49cece15f6dccdea58eef0157e4f053
https://pure.eur.nl/en/publications/77148a37-8e09-4607-b8b4-2ebf267fbb6f

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Deletion of the Kv2.1 delayed rectifier potassium channel leads to neuronal and behavioral hyperexcitability

Autor: Steve W. Wiler, Genki Ogata, Danielle Mandikian, Jeanne M. Nerbonne, Hannah I. Bishop, Lucas Matt, H. Jürgen Wenzel, Johannes W. Hell, Katharine L. Campi, Philip A. Schwartzkroin, Jon T. Sack, Brian C. Trainor, James S. Trimmer, Emily T. Doisy, David J. Speca, Mari S. Golub, Kenneth S. Eum

Publikováno v: Genes, Brain and Behavior. 13:394-408

The Kv2.1 delayed rectifier potassium channel exhibits high-level expression in both principal and inhibitory neurons throughout the central nervous system, including prominent expression in hippocampal neurons. Studies of in vitro preparations sugge

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::3e7f98b4ac6546a1337bd06df68237d2
https://doi.org/10.1111/gbb.12120

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Are developmental dysplastic lesions epileptogenic?

Autor: Philip A. Schwartzkroin, H. Jürgen Wenzel

Publikováno v: Epilepsia. 53:35-44

Summary Cortical dysplasia of various types, reflecting abnormalities of brain development, have been closely associated with epileptic activities. Yet, there remains considerable discussion about if/how these structural lesions give rise to seizure

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::1a06f757a015646ab958efa6ea6a07e2
https://doi.org/10.1111/j.1528-1167.2012.03473.x

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Acute, but reversible, kainic acid-induced DNA damage in hippocampal CA1 pyramidal cells of p53-deficient mice

Autor: Yoshito Kinoshita, Chizuru Kinoshita, Richard S. Morrison, H. Jürgen Wenzel, Philip A. Schwartzkroin

Publikováno v: Epilepsia. 53:125-133

Summary p53 plays an essential role in mediating apoptotic responses to cellular stress, especially DNA damage. In a kainic acid (KA)–induced seizure model in mice, hippocampal CA1 pyramidal cells undergo delayed neuronal death at day 3–4 followi

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::8b08b165bbb08ae6741ebb0df0e36f07
https://doi.org/10.1111/j.1528-1167.2012.03483.x

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Inhibition and interneuron distribution in the dentate gyrus of p35 knockout mice

Autor: Philip A. Schwartzkroin, H. Jürgen Wenzel, Leena S. Knight

Publikováno v: Epilepsia. 53:161-170

The p35 knockout (p35-/-) mouse is an animal model of temporal lobe epilepsy that recapitulates key neuroanatomic abnormalities-granule cell dispersion and mossy fiber sprouting-observed in the hippocampal formation of humans, as well as spontaneous

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::54e573de7a03e912f68d50cf7b8e4d3b
https://doi.org/10.1111/j.1528-1167.2012.03487.x

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Abnormal dendrite and spine morphology in primary visual cortex in the CGG knock-in mouse model of the fragile X premutation

Autor: Robert F. Berman, Karl D Murray, Michael R. Hunsaker, H. Jürgen Wenzel, Gloria Arque

Publikováno v: Epilepsia. 53:150-160

The fragile X mental retardation 1 gene (Fmr1) is polymorphic for CGG trinucleotide repeat number in the 5'-untranslated region, with repeat lengths 200 resulting in hypermethylation and transcriptional silencing of the gene and mental retardation in

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::4fcf1ea1f9bfea76502ba10c3b75aa84
https://doi.org/10.1111/j.1528-1167.2012.03486.x

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Initiation of epileptiform activity in a rat model of periventricular nodular heterotopia

Autor: H. Jürgen Wenzel, Emily T. Doisy, Naranzogt Tschuluun, Philip A. Schwartzkroin

Publikováno v: Epilepsia. 52:2304-2314

Purpose Periventricular nodular heterotopia (PNH) are, in humans, often associated with difficult-to-control epilepsy. However, there is considerable controversy about the role of the PNH in seizure generation and spread. To study this issue, we have

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::1d8f43ba043bd69b7a986152106c7954
https://doi.org/10.1111/j.1528-1167.2011.03264.x

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Progressive Spatial Processing Deficits in a Mouse Model of the Fragile X Premutation

Autor: Rob Willemsen, H. Jürgen Wenzel, Michael R. Hunsaker, Robert F. Berman

Publikováno v: Behavioral Neuroscience, 123(6), 1315-1324. American Psychological Association Inc.

Fragile X associated tremor/ataxia syndrome (FXTAS) is a neurodegenerative disorder that is the result of a CGG trinucleotide repeat expansion in the range of 55-200 in the 5' UTR of the FMR1 gene. To better understand the progression of this disorde

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c9e9e5ba0f461ff3181896b79834ef4f
https://pure.eur.nl/en/publications/0a8c9266-1d0f-4f82-a289-85bed339e6c1

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Nodule excitability in an animal model of periventricular nodular heterotopia: c-fos activation in organotypic hippocampal slices

Autor: H. Jürgen Wenzel, Danh V. Nguyen, Philip A. Schwartzkroin, Yi Mu, Emily T. Doisy

Publikováno v: Doisy, Emily T; Wenzel, H. Jürgen; Mu, Yi; Nguyen, Danh V; & Schwartzkroin, Philip A. (2015). Nodule excitability in an animal model of periventricular nodular heterotopia: c-fos activation in organotypic hippocampal slices. Epilepsia, 56(4), 626-635. doi: 10.1111/epi.12945. UC Irvine: Institute for Clinical and Translational Science. Retrieved from: http://www.escholarship.org/uc/item/8f3770jn
Epilepsia, vol 56, iss 4
Doisy, ET; Wenzel, HJ; Mu, Y; Nguyen, DV; & Schwartzkroin, PA. (2015). Nodule excitability in an animal model of periventricular nodular heterotopia: C-fos activation in organotypic hippocampal slices. Epilepsia, 56(4), 626-635. doi: 10.1111/epi.12945. UC Irvine: Retrieved from: http://www.escholarship.org/uc/item/5f47f30q

© Wiley Periodicals, Inc. © 2015 International League Against Epilepsy. Summary Objective Aberrations in brain development may lead to dysplastic structures such as periventricular nodules. Although these abnormal collections of neurons are often a

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::83df6633075efc73396d51523c5ef536
http://www.escholarship.org/uc/item/8f3770jn

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