Neurophysiology of neurodegenerative diseases

Group leader: Filippo Tempia

Publications

2022

Claudia Cristiano, Eriola Hoxha, Pellegrino Lippiello, Ilaria Balbo, Roberto Russo, Filippo Tempia, Maria Concetta Miniaci (2022) Maternal treatment with sodium butyrate reduces the development of autism-like traits in mice offspring. Biomedicine & Pharmacotherapy 156: 113870. https://doi.org/10.1016/j.biopha.2022.113870

Balbo I, Montarolo F, Genovese F, Tempia F, Hoxha E. Effects of the administration of Elovl5-dependent fatty acids on a spino-cerebellar ataxia 38 mouse model Behav Brain Funct 18, 8 (2022). https://doi.org/10.1186/s12993-022-00194-4

Montarolo, Francesca; Martire, Serena; Francesco, Chiara; Allegra, Sarah; Silvia, De Francia; Hoxha, Eriola; Tempia, Filippo; Alfonso, Capobianco Marco; Bertolotto, Antonio (2022) NURR1 deficient mice have age- and sex-specific behavioral phenotypes. J Neurosci Res 100:1747-1754. https://doi.org/10.1002/jnr.25067

2021

Eriola Hoxha, Ilaria Balbo, Roberta Parolisi, Matteo Audano, Francesca Montarolo, Francesco Ravera, Michela Guglielmotto, Luisa Muratori, Stefania Raimondo, Eleonora Di Gregorio, Annalisa Buffo, Alfredo Brusco, Barbara Borroni, Nico Mitro, Donatella Caruso, Filippo Tempia (2021) Elovl5 is required for proper action potential conduction along peripheral myelinated fibers. Glia. 17 June 2021 https://doi.org/10.1002/glia.24048

Ilaria Balbo, Francesca Montarolo, Enrica Boda, Filippo Tempia and Eriola Hoxha (2021) Elovl5 Expression in the Central Nervous System of the Adult Mouse. Front. Neuroanat., 29 April 2021 doi.org/10.3389/fnana.2021.669073

2020

Lippiello P, Hoxha E, Tempia F, Miniaci MC. (2020) GIRK1-mediated inwardly rectifying potassium current is a candidate mechanism behind Purkinje cell excitability, plasticity and neuromodulation. Cerebellum, Online First 02 July 2020. https://doi.org/10.1007/s12311-020-01158-y. Lippiello, Pellegrino; Hoxha, Eriola; Cristiano, Claudia; Malvicini, Emilia;

Stanley, Adrien; Russo, Roberto; Tempia, Filippo; Miniaci, Maria. (2020) Role of β3-adrenergic receptor in the modulation of synaptic transmission and plasticity in mouse cerebellar cortex. J Neuroscie Res 98: 2263-2274. https://doi.org/10.1002/jnr.24712.

2019

Mancini C, Hoxha E, Iommarini L, Brussino A, Richter U, Montarolo F, Cagnoli C, Parolisi R, Gondor Morosini DI, Nicolò V, Maltecca F, Muratori L, Ronchi G, Geuna S, Arnaboldi F, Donetti E, Giorgio E, Cavalieri S, Di Gregorio E, Pozzi E, Ferrero M, Riberi E, Casari G, Altruda F, Turco E, Gasparre G, Battersby BJ,Porcelli AM, Ferrero E, Brusco A, Tempia F. Mice harbouring a SCA28 patient mutation in AFG3L2 develop late-onset ataxia associated with enhanced mitochondrial proteotoxicity. Neurobiol Dis. 2019 Apr;124:14-28. doi: 10.1016/j.nbd.2018.10.018. Epub 2018 Oct 30. PubMed PMID: 30389403.

Hoxha E, Marcinnò A, Montarolo F, Masante L, Balbo I, Laezza F, Tempia F (2019) Emergingroles of Fgf14 in behavioral control. Behavioral Brain Research 356: 257-265.

Manes M, Alberici A, Di Gregorio E, Boccone L, Premi E, Mitro N, Pasolini MP,Pani C, Paghera B, Orsi L, Costanzi C, Ferrero M, Tempia F, Caruso D, Padovani A, Brusco A, Borroni B (2019) Long-termefficacy of Docosahexaenoic acid (DHA) for SpinocerebellarAtaxia 38 (SCA38) treatment: an open labelextension study. Parkinsonism & RelatedDisorders.

2018

Hoxha E, Lippiello P, Zurlo F, Balbo I, Santamaria R, Tempia F, Miniaci MC. The Emerging Role of Altered Cerebellar Synaptic Processing in Alzheimer's Disease. Front Aging Neurosci. 2018 Nov 27;10:396. eCollection 2018. Review.

Hoxha E, Balbo I, Miniaci MC, Tempia F (2018) Purkinje cell signaling deficits in animal models of ataxia . Front. Synaptic Neurosci. 10:6 (17 pages). eCollection 2018. Review. http://dx.doi.org/10.3389/fnsyn.2018.00006.

Cerrato V, Mercurio S, Leto K, Fucà E, Hoxha E, Bottes S, Pagin M, Milanese M, Ngan C-Y, Concina G, Ottolenghi S, Wei C-L, Bonanno G, Pavesi G, Tempia F, Buffo A, Nicolis SK (2018) Sox2 conditional mutation in mouse causes ataxic symptoms, cerebellar vermis hypoplasia, and postnatal defects of Bergmann glia. Glia. http://dx.doi.org/10.1002/glia.23448 .

Eriola Hoxha*, Andrea Marcinnò*, Francesca Montarolo, Linda Masante, Ilaria Balbo, Fernanda Laezza, Filippo Tempia (2018) Emerging roles of Fgf14 in behavioral control. Behavioral Brain Research 356: 257-265. https://doi.org/10.1016/j.bbr.2018.08.034.

Cecilia Mancini*, Eriola Hoxha*, Luisa Iommarini, Alessandro Brussino, Uwe Richter, Francesca Montarolo, Claudia Cagnoli, Roberta Parolisi, Diana Iulia Gondor Morosini, Valentina Nicolò, Francesca Maltecca, Luisa Muratori, Giulia Ronchi, Stefano Geuna, Francesca Arnaboldi, Elena Donetti, Elisa Giorgio, Simona Cavalieri, Eleonora Di Gregorio, Elisa Pozzi, Marta Ferrero, Evelise Riberi, Giorgio Casari, Fiorella Altruda, Emilia Turco, Giuseppe Gasparre, Brendan J. Battersby, Anna Maria Porcelli, Alfredo Brusco*, Filippo Tempia* (2018) Mice harbouring a SCA28 patient mutation in AFG3L2 develop late-onset ataxia associated with enhanced mitochondrial proteotoxicity. Neurobiol Dis. 2018 Oct 30. pii: S0969-9961(18)30320-6. doi: 10.1016/j.nbd.2018.10.018. *contributed equally

2017

Hoxha E, Gabriele RMC, Balbo I, Ravera F, Masante L, Zambelli V, Albergo C, Mitro N, Caruso D, Di Gregorio E, Brusco A, Borroni B, Tempia F. Motor Deficits and Cerebellar Atrophy in Elovl5 Knock Out Mice. Front Cell Neurosci. 2017 Oct 30;11:343. doi: 10.3389/fncel.2017.00343. eCollection 2017.

Hoxha E, Lippiello P, Scelfo B, Tempia F, Ghirardi M, Miniaci MC. (2017) Maturation and refinement of cerebellar cortical circuits in normal development and in murine models of autism . Neural Plasticity, Article ID 2017:6595740, 14 pages. Review. doi: 10.1155/2017/6595740.

Manes M, Alberici A, Di Gregorio E, Boccone L, Premi E, Mitro N, Pasolini MP, Pani C, Paghera B, Perani D, Orsi L, Costanzi C, Ferrero M, Zoppo A, Tempia F, Caruso D, Grassi M, Padovani A, Brusco A, Borroni B (2017) DHA is a beneficial replacement treatment for Spinocerebellar Ataxia 38 (SCA38) . Ann Neurol. 2017 Oct 4. doi: 10.1002/ana.25059.

2016

Eriola Hoxha, Filippo Tempia, Pellegrino Lippiello, Maria Concetta Miniaci. (2016) Modulation, plasticity and pathophysiology of the parallel fiber-Purkinje cell synapse . Frontiers in Synaptic Neuroscience 8:35; doi: http://dx.doi.org/10.3389/fnsyn.2016.00035

Alshammari TK,Alshammari MA, Nenov MN, Hoxha E, Cambiaghi M, Marcinno A, James TF, Singh P, Labate D, Li J, Meltzer HY, Sacchetti B, Tempia F, Laezza F. Genetic deletion of fibroblast growth factor 14 recapitulates phenotypic alterations underlying cognitive impairment associated with schizophrenia . Transl Psychiatry (2016) 6, e806; http://dx.doi.org/10.1038/tp.2016.66

Barbara Borroni, Eleonora Di Gregorio, Laura Orsi, Giovanna Vaula, Chiara Costanzi, Filippo Tempia, Nico Mitro, Donatella Caruso, Marta Manes, Lorenzo Pinessi, Alessandro Padovani, Alfredo Brusco, Loredana Boccone (2016) Clinical and neuroradiological features of spinocerebellar ataxia 38 (SCA38) . Parkinsonism & Related Disorders; http://dx.doi.org/10.1016/j.parkreldis.2016.04.030

Cupolillo D, Hoxha E, Faralli A, De Luca A, Tempia F, Rossi F, Carulli D (2016) Autistic-like traits and cerebellar dysfunction in Purkinje cell PTEN knock-out mice. Neuropsychopharmacol doi: 10.1038/npp.2015.339

Lippiello Pellegrino, Hoxha Eriola, Speranza Luisa, Volpicelli Floriana, Ferraro Angela, Leopoldo Marcello, Lacivita Enza, Perrone-Capano Carla, Tempia Filippo and Miniaci Maria Concetta. (2016) The 5-HT7 Receptor Triggers Cerebellar Long-Term Synaptic Depression via PKC-MAPK . J Neuropharmacol 101: 426-438. http://dx.doi.org/10.1016/j.neuropharm.2015.10.019

2015

Sadallah M, Labat-Gest V, Tempia F. Propagation of neuronal damage to embryonic grafts transplanted in the hippocampus of murine models of Alzheimer's disease. Rejuvination Res. (Instant Online ahead of print June 2, 2015)

Tempia F, Hoxha E, Negro G, Alshammari MA, Alshammari TK, Panova-Elektronova N and Laezza F (2015) Parallel fiber to Purkinje cell synaptic impairment in a mouse model of spinocerebellar ataxia type 27 . Front. Cell. Neurosci. 9:205.

Sallam HS, Tumurbaatar B, Zhang WR, Tuvdendorj D, Chandalia M, Tempia F, Laezza F, Taglialatela G, Abate N. (2015) Peripheral adipose tissue insulin resistance alters lipid composition and function of hippocampal synapses. J Neurochem 133: 125-133.

Nenov MN, Tempia F, Denner L, Dineley KT, Laezza F. (2015) Impaired firing properties of dentate granule neurons in an Alzheimer's disease animal model are rescued by PPARγ agonism . J Neurophysiol 113: 1712-1726.

Lippiello P, Hoxha E, Volpicelli F, Lo Duca G, Tempia F*, Miniaci MC. (2015) Noradrenergic Modulation of the Parallel Fiber-Purkinje Cell Synapse in Mouse Cerebellum. Neuropharmacol 89: 33-42. DOI: 10.1016/j.neuropharm.2014.08.016. (* corresponding author)

2014

Di Gregorio E, Borroni B, Giorgio E, Lacerenza D, Ferrero M, Lo Buono N, Ragusa N, Mancini C, Gaussen M, Calcia A, Mitro N, Hoxha E, Mura I, Coviello DA, Moon YA, Tesson C, Vaula G, Couarch P, Orsi L, Duregon E, Papotti MG, Deleuze JF, Imbert J, Costanzi C, Padovani A, Giunti P, Maillet-Vioud M, Durr A, Brice A, Tempia F, Funaro A, Boccone L, Caruso D, Stevanin G, Brusco A. (2014) ELOVL5 Mutations Cause Spinocerebellar Ataxia 38. Am J Hum Genet 95: 209-217. pii: S0002-9297(14)00310-3. doi: 10.1016/j.ajhg.2014.07.001.

Lippiello P, Hoxha E, Volpicelli F, De Luca G, Tempia F, Miniaci MC (2014). Noradrenergic Modulation of the Parallel Fiber-Purkinje Cell Synapse in Mouse Cerebellum. Neuropharmacol 89: 33-42

2013

Montarolo F, Parolisi R, Hoxha E, Boda E, Tempia F (2013) Early enriched environment exposure protects spatial memory and accelerates amyloid plaque formation in APPSwe/PS1L166P mice. PLoS ONE 8(7): e69381. (doi:10.1371/journal.pone.0069381). IF: 3.730

Di Gregorio E, Bianchi FT, Schiavi A, Chiotto AM, Rolando M, Verdun di Cantogno L, Grosso E, Cavalieri S, Calcia A, Lacerenza D, Zuffardi O, Retta SF, Stevanin G, Marelli C, Durr A, Forlani S, Chelly J, Montarolo F, Tempia F, Beggs HE, Reed R, Squadrone S, Abete MC, Brussino A, Ventura N, Di Cunto F, Brusco A. (2013) A de novo X;8 translocation creates a PTK2-THOC2 gene fusion with THOC2 expression knockdown in a patient with psychomotor retardation and congenital cerebellar hypoplasia. J Med Genet. 50: 543-551. (doi:10.1136/jmedgenet-2013-101542). IF: 5.703

Hoxha E, Tonini R, Montarolo F, Croci L, Consalez GG, Tempia F (2013) Motor dysfunction and cerebellar Purkinje cell firing impairment in Ebf2 null mice. Mol Cell Neurosci 52: 51-61. (10.1016/j.mcn.2012.09.002).

Sadallah M, Tempia F (2013) Spreading of amyloidosis to unaffected areas of the hippocampus studied in vivo by nervous tissue transplantation. 43rd annual meeting of the Society for Neuroscience, San Diego, CA, USA, November 9-13, 2013. Abstract

2012

Hoxha E, Boda E, Montarolo F, Parolisi R, Tempia F (2012) Excitability and synaptic alterations in the cerebellum of APP/PS1 mice. PLoS ONE 7(4): e34726 (13 pages) (doi:10.1371/journal.pone.0034726).

Boda E*, Hoxha E*, Pini A, Montarolo F, Tempia F (2012) Brain expression of Kv3 subunits during development, adulthood, aging and in a murine model of Alzheimer's disease. J Mol Neurosci 46: 606-615 (doi: 10.1007/s12031-011-9648-6).

2011

Boda E, Viganò F, Rosa P, Fumagalli M, Labat-gest V, Tempia F, Abbracchio MP, Dimou L, Buffo A. (2011) The GPR17 receptor in NG2 expressing cells: focus on in vivo cell maturation and participation in acute trauma and chronic damage. Glia 59:1958-1973 (doi: 10.1002/glia.21237).

Bianchi FT, Camera P, Ala U, Imperiale D, Migheli A, Boda E, Tempia F, Berto G, Bosio Y, Oddo S, LaFerla FM, Taraglio S, Dotti CG, Di Cunto F (2011) The collagen chaperone HSP47 is a new interactor of APP that affects the levels of extracellular beta-amyloid peptides. PLoS ONE 6(7): e22370 (13 pages) (doi:10.1371/journal.pone.0022370).

2010

Sacco T*, Boda E*, Hoxha E, Pizzo R, Cagnoli C, Brusco A, Tempia F. (2010) Mouse brain expression patterns of Spg7, Afg3l1, and Afg3l2 transcripts, encoding for the mitochondrial m-AAA protease. BMC Neuroscience 11: 55 (doi:10.1186/1471-2202-11-55).

Di Bella D, Lazzaro F, Brusco A, Plumari M, Battaglia G, Pastore A, Finardi A, Cagnoli C, Tempia F, Frontali M, Veneziano L, Sacco T, Boda E, Brussino A, Bonn F, Castellotti B, Baratta S, Mariotti C, Gellera C, Fracasso V, Magri S, Langer T, Plevani P, Di Donato S, Muzi-Falconi M, Taroni F. (2010) Mutations in the mitochondrial protease gene AFG3L2 cause dominant hereditary ataxia SCA28. Nature Genetics 42: 313-331 (doi:10.1038/ng.544).

Before 2010

Boda E, Pini A, Hoxha E, Parolisi R, Tempia F. (2009) Selection of reference genes for quantitative real time rt-pcr studies in mouse brain. J Mol Neurosci 37: 238-253 (doi: 10.1007/s12031-008-9128-9).

Sacco T, De Luca A, Tempia F. (2006) Properties and expression of Kv3 channels in cerebellar Purkinje cells. Mol Cell Neurosci 33: 170-179.

Schiavon E*, Sacco T*, Restano Cassulini R, Gurrola G, Tempia F, Possani LD, and Wanke E (2006) Resurgent current and voltage sensor-trapping enhanced activation by a ß-scorpion toxin solely in Nav1.6 channel: significance in mice Purkinje neurons. J Biol Chem 281: 20326-20337.

Guasti L, Cilia E, Crociani O, Hofmann G, Polvani S, Becchetti A, Wanke E, Tempia F, Arcangeli A. (2005) Expression pattern of the ether-a-go-go-related (ERG) family proteins in the adult mouse central nervous system: evidence for coassembly of different subunits. J Comp Neurol 491:157-174.

Sacchetti B, Scelfo B, Tempia F, Strata P. (2004) Long-term synaptic changes induced in the cerebellar cortex by fear conditioning. Neuron 42: 973-982.

Sacco T., Bruno A., Wanke E., Tempia F. (2003) Functional roles of an ERG current isolated in cerebellar Purkinje neurons. J Neurophysiol 90: 1817-1828.

Sacco T., Tempia F. (2002) A-type potassium currents active at subthreshold potentials in mouse cerebellar Purkinje cells. J Physiol 543: 505-520.

Anchisi D., Scelfo B., Tempia F. (2001) Postsynaptic currents in deep cerebellar nuclei. J Neurophysiol 85: 323-331.
Tempia F., Alojado M.E., Strata P., Knopfel T. (2001) Characterization of the mGluR1- mediated electrical and calcium signaling in Purkinje cells of mouse cerebellar slices. J Neurophysiol 86: 1389-1397.

Knopfel T., Anchisi D., Alojado M.E., Tempia F., Strata P. (2000) Elevation of intradendritic sodium concentration mediated by synaptic activation of metabotropic glutamate receptors in cerebellar Purkinje cells. Eur J Neurosci 12: 2199-2204.

Chiesa N., Barlow C., Wynshaw-Boris A., Strata P., Tempia F. (2000) Atm-deficient mice Purkinje cells show age dependent defects in calcium spike bursts and calcium currents. Neuroscience 96: 575-583.

Tempia F., Miniaci M.C., Anchisi D., Strata P. (1998) Postsynaptic current mediated by metabotropic glutamate receptors in cerebellar Purkinje cells. J Neurophysiol 80: 520-528.

Strata F., Atzori M., Molnar M., Ugolini G., Tempia F., Cherubini E. (1997) A pacemaker current in dye coupled hilar interneurons contributes to the generation of giant GABAergic potentials in developing hippocampus. J. Neurosci. 17: 1435-1446.

Tempia F., Bravin M., Strata P. (1996) Postsynaptic currents and short-term synaptic plasticity in Purkinje cells grafted onto an adult cerebellar cortex. Eur. J. Neurosci. 8: 2690-2701.

Garaschuk O., Schneggenburger R., Schirra C., Tempia F., Konnerth A. (1996) Fractional calcium currents through somatic and dendritic glutamate receptor channels of hippocampal CA1 pyramidal neurones. J. Physiol. (London) 491: 757-772.

Tempia F., Kano M., Schneggenburger R., Schirra C., Garaschuk O., Plant T., Konnerth A. (1996) T he fractional calcium current through neuronal AMPA receptor channels with a 'low' calcium permeability. J. Neurosci. 16: 456-466.

Schneggenburger R., Tempia F., Konnerth A. (1993) Glutamate- and AMPA-mediated calcium influx through glutamate receptors channels in medial septal neurons. Neuropharmacol. 32: 1221-1228.

Tempia F., Ghirardi M., Dotta M., Strata P. (1992) Spontaneous gaze shifts in intact head-free rats and following inferior olive and cerebellar lesions. Eur. J. Neurosci. 4: 1239-1248.

Tempia F., Dieringer N., Strata P. (1991) Adaptation and habituation of the vestibulo-ocular reflex in intact and inferior olive-lesioned rats. Exp. Brain Res. 86: 568-578.

Chelazzi L., Ghirardi M., Rossi F., Strata P., Tempia F. (1990). Spontaneous saccades and gaze-holding ability in the pigmented rat. II. Effects of localized cerebellar lesions. Eur. J. Neurosci. 2: 1085-1094.

Savio T., Tempia F. (1985). On the Purkinje cell activity increase induced by suppression of inferior olive activity. Exp. Brain Res. 57: 456-563.

Chelazzi L., Rossi F., Tempia F., Ghirardi M., Strata P. (1989). Saccadic eye movements and gaze holding in the head-restrained pigmented rat. Eur. J. Neurosci. 1: 639-646.

Benedetti F., Montarolo P.G., Strata P., Tempia F. (1983). Inferior olive inactivation decreases the excitability of the intracerebellar and lateral vestibular nuclei in the rat. J. Physiol. (London) 340: 195-208.

Neurophysiology of neurodegenerative diseases

Publications

2022

2021

2020

2019

2018

2017

2016

2015

2014

2013

2012

2011

2010

Before 2010

Physiopathology of neural stem cells

Neurophysiology of neurodegenerative diseases

Brain development and disease

Aging and Alzheimer’s Disease

Clinical neurobiology

Adult neurogenesis

Neuropsychopharmacology

Nerve regeneration

Neuroendocrinology

Embryonic neurogenesis