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Purkinje cell differentiation ipsc

Hiroyuki Morino, Yukiko Matsuda, Keiko Muguruma, Ryosuke Miyamoto, Ryosuke Ohsawa, Toshiyuki Ohtake, Reiko Otobe, Masahiko Watanabe, Hirofumi Maruyama, Kouichi Hashimoto, Hideshi Kawakami
BACKGROUND: Spinocerebellar ataxia (SCA) is a genetically heterogeneous disease. To date, 36 dominantly inherited loci have been reported, and 31 causative genes have been identified. RESULTS: In this study, we analyzed a Japanese family with autosomal dominant SCA using linkage analysis and exome sequencing, and identified CACNA1G, which encodes the calcium channel CaV3.1, as a new causative gene. The same mutation was also found in another family with SCA. Although most patients exhibited the pure form of cerebellar ataxia, two patients showed prominent resting tremor in addition to ataxia...
2015: Molecular Brain
Shuyan Wang, Bin Wang, Na Pan, Linlin Fu, Chaodong Wang, Gongru Song, Jing An, Zhongfeng Liu, Wanwan Zhu, Yunqian Guan, Zhi-Qing David Xu, Piu Chan, Zhiguo Chen, Y Alex Zhang
It remains a challenge to differentiate human induced pluripotent stem cells (iPSCs) or embryonic stem (ES) cells to Purkinje cells. In this study, we derived iPSCs from human fibroblasts and directed the specification of iPSCs first to Purkinje progenitors, by adding Fgf2 and insulin to the embryoid bodies (EBs) in a time-sensitive manner, which activates the endogenous production of Wnt1 and Fgf8 from EBs that further patterned the cells towards a midbrain-hindbrain-boundary tissue identity. Neph3-positive human Purkinje progenitors were sorted out by using flow cytometry and cultured either alone or with granule cell precursors, in a 2-dimensional or 3-dimensional environment...
2015: Scientific Reports
Marion Najac, Indira M Raman
Neurons in the cerebellar cortex, cerebellar nuclei, and inferior olive (IO) form a trisynaptic loop critical for motor learning. IO neurons excite Purkinje cells via climbing fibers and depress their parallel fiber inputs. Purkinje cells inhibit diverse cells in the cerebellar nuclei, including small GABAergic nucleo-olivary neurons that project to the IO. To investigate how these neurons integrate synaptic signals from Purkinje cells, we retrogradely labeled nucleo-olivary cells in the contralateral interpositus and lateral nuclei with cholera toxin subunit B-Alexa Fluor 488 and recorded their electrophysiological properties in cerebellar slices from weanling mice...
January 14, 2015: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
Zoé Husson, Charly V Rousseau, Ilja Broll, Hanns Ulrich Zeilhofer, Stéphane Dieudonné
The principal neurons of the cerebellar nuclei (CN), the sole output of the olivo-cerebellar system, receive a massive inhibitory input from Purkinje cells (PCs) of the cerebellar cortex. Morphological evidence suggests that CN principal cells are also contacted by inhibitory interneurons, but the properties of this connection are unknown. Using transgenic, tracing, and immunohistochemical approaches in mice, we show that CN interneurons form a large heterogeneous population with GABA/glycinergic phenotypes, distinct from GABAergic olive-projecting neurons...
July 9, 2014: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
Shunsuke Toyoda, Masahumi Kawaguchi, Toshihiro Kobayashi, Etsuko Tarusawa, Tomoko Toyama, Masaki Okano, Masaaki Oda, Hiromitsu Nakauchi, Yumiko Yoshimura, Makoto Sanbo, Masumi Hirabayashi, Teruyoshi Hirayama, Takahiro Hirabayashi, Takeshi Yagi
In the brain, enormous numbers of neurons have functional individuality and distinct circuit specificities. Clustered Protocadherins (Pcdhs), diversified cell-surface proteins, are stochastically expressed by alternative promoter choice and affect dendritic arborization in individual neurons. Here we found that the Pcdh promoters are differentially methylated by the de novo DNA methyltransferase Dnmt3b during early embryogenesis. To determine this methylation's role in neurons, we produced chimeric mice from Dnmt3b-deficient induced pluripotent stem cells (iPSCs)...
April 2, 2014: Neuron
Katrin Streckfuss-Bömeke, Frieder Wolf, Azadeh Azizian, Michael Stauske, Malte Tiburcy, Stefan Wagner, Daniela Hübscher, Ralf Dressel, Simin Chen, Jörg Jende, Gerald Wulf, Verena Lorenz, Michael P Schön, Lars S Maier, Wolfram H Zimmermann, Gerd Hasenfuss, Kaomei Guan
AIMS: Induced pluripotent stem cells (iPSCs) provide a unique opportunity for the generation of patient-specific cells for use in disease modelling, drug screening, and regenerative medicine. The aim of this study was to compare human-induced pluripotent stem cells (hiPSCs) derived from different somatic cell sources regarding their generation efficiency and cardiac differentiation potential, and functionalities of cardiomyocytes. METHODS AND RESULTS: We generated hiPSCs from hair keratinocytes, bone marrow mesenchymal stem cells (MSCs), and skin fibroblasts by using two different virus systems...
September 2013: European Heart Journal
Melissa H Kelley, Masayuki Kuroiwa, Noriko Taguchi, Paco S Herson
Allopregnanolone (ALLO) is a neurosteroid that has many functions in the brain, most notably neuroprotection and modulation of gamma-amino butyric acid (GABA) neurotransmission. Using a mouse model of cardiac arrest and cardiopulmonary resuscitation, we have previously demonstrated that ALLO protects cerebellar Purkinje cells (PCs) from ischemia in a GABA(A) receptor-dependent manner. In this study we examined the effect of sex on ALLO neuroprotection, observing that low dose ALLO (2 mg/kg) provided greater neuroprotection in females compared to males...
September 2011: Neuropharmacology
Manuel Mameli, Paolo Botta, Paula A Zamudio, Stefano Zucca, C Fernando Valenzuela
Cerebellar Purkinje neurons (PNs) receive inhibitory GABAergic input from stellate and basket cells, which are located in the outer and inner portions of the molecular layer, respectively. Ethanol (EtOH) was recently shown to increase GABAergic transmission at PNs via a mechanism that involves enhanced calcium release from presynaptic internal stores (J Pharmacol Exp Ther 323:356-364, 2007). Here, we further characterized the effect of EtOH on GABA release and assessed its impact on PN excitability. Using whole-cell patch-clamp electrophysiological techniques in cerebellar vermis parasagittal slices, we found that EtOH acutely increases the frequency but not the amplitude or half-width of miniature and spontaneous inhibitory postsynaptic currents (IPSCs)...
December 2008: Journal of Pharmacology and Experimental Therapeutics
Jean-Marc Fritschy, Patrizia Panzanelli, Jason E Kralic, Kaspar E Vogt, Marco Sassoè-Pognetto
Synapse formation and maintenance require extensive transsynaptic interactions involving multiple signal transduction pathways. In the cerebellum, Purkinje cells (PCs) receive GABAergic, axo-dendritic synapses from stellate cells and axo-somatic synapses from basket cells, both with GABAA receptors containing the alpha1 subunit. Here, we investigated the effects of a targeted deletion of the alpha1 subunit gene on GABAergic synaptogenesis in PCs, using electrophysiology and immunoelectron microscopy. Whole-cell patch-clamp recordings in acute slices revealed that PCs from alpha1(0/0) mice lack spontaneous and evoked IPSCs, demonstrating that assembly of functional GABAA receptors requires the alpha1 subunit...
March 22, 2006: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
Yukun Yuan, William D Atchison
Using whole-cell recording techniques we compared effects of the environmental cerebellar neurotoxicant methylmercury (MeHg) on spontaneous IPSCs (sIPSCs) of both Purkinje and granule cells in cerebellar slices of the rat. In Purkinje cells, bath application of 10, 20 or 100 microM MeHg initially increased then suppressed the frequency of sIPSCs to zero. In granule cells, the initial increase in frequency was not observed in approximately 50% of cells examined, but suppression of sIPSCs by MeHg occurred in every cell tested...
July 1, 2003: Journal of Physiology
H Mitoma, T Kobayashi, S Y Song, S Konishi
The actions of serotonin (5-HT) on synaptic responses in Purkinje cells were examined using the whole-cell voltage-clamp recording from thin slices of the rat cerebellum. Focal stimulation within the molecular layer elicited synaptic responses: (1) inhibitory postsynaptic currents (IPSCs) that were completely blocked by bicuculline; (2) excitatory postsynaptic currents (EPSCs) that were antagonized by CNQX; and (3) combination of IPSC and EPSC. Application of 5-HT (3-30 microM) increased the amplitude of the stimulation-evoked IPSCs but did not significantly affect the EPSCs...
May 23, 1994: Neuroscience Letters
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