Dr. Jeremie Vitte

Head and Neck Surgery, University of California, Los Angeles, Los Angeles, California, USA

Dr. Jeremie Vitte

Head and Neck Surgery, University of California, Los Angeles, Los Angeles, California, USA

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Academic Career Length

23Years (2004-Present)

(Calculated once Scilit publication data is synced: current year − year of first publication + 1)

Representative Publications

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13 November 2006 in Human Molecular Genetics

Journal Article

A mutation of spastin is responsible for swellings and impairment of transport in a region of axon characterized by changes in microtubule composition

Anne Tarrade

Sabrina Courageot

Mutations of the spastin gene (Sp) are responsible for the most frequent autosomal dominant form of spastic paraplegia, a disease characterized by the degeneration of corticospinal tracts. We show that a deletion in the mouse Sp gene, generating a premature stop codon, is responsible for progressive axonal degeneration, restricted to the central nervous system, leading to a late and mild motor defect. The degenerative process is characterized by focal axonal swellings, associated with abnormal accumulation of organelles and cytoskeletal components. In culture, mutant cortical neurons showed normal viability and neurite density. However, they develop neurite swellings associated with focal impairment of retrograde transport. These defects occur near the growth cone, in a region characterized by the transition between stable microtubules rich in detyrosinated α-tubulin and dynamic microtubules composed almost exclusively of tyrosinated α-tubulin. Here, we show that the Sp mutation has a major impact on neurite maintenance and transport both in vivo and in vitro. These results highlight the link between spastin and microtubule dynamics in axons, but not in other neuronal compartments. In addition, it is the first description of a human neurodegenerative disease which involves this specialized region of the axon.

7 February 2013 in American Journal of Medical Genetics Part A

Journal Article

Update from the 2011 International Schwannomatosis Workshop: From genetics to diagnostic criteria

Scott Plotkin

Jaishri O. Blakeley

D. Gareth Evans

C. Oliver Hanemann

Theo J.M. Hulsebos

Marco Giovannini

+10

1 October 2007 in The American Journal of Pathology

Journal Article

Refined Characterization of the Expression and Stability of the SMN Gene Products

Jeremie Vitte

Coralie Fassier

Francesco D. Tiziano

Cécile Dalard

Sabrina Soave

Judith Melki

1 November 2004 in The American Journal of Pathology

Journal Article

Deletion of Murine Smn Exon 7 Directed to Liver Leads to Severe Defect of Liver Development Associated with Iron Overload

Jeremie Vitte

Bénédicte Davoult

Natacha Roblot

Michèle Mayer

Vandana Joshi

Judith Melki

21 August 2017 in Nature Communications

Journal Article

Timing of Smarcb1 and Nf2 inactivation determines schwannoma versus rhabdoid tumor development

Jeremie Vitte

Fuying Gao

Giovanni Coppola

Alexander R. Judkins

Marco Giovannini

Germline mutations of the SMARCB1 gene predispose to two distinct tumor syndromes: rhabdoid tumor predisposition syndrome, with malignant pediatric tumors mostly developing in brain and kidney, and familial schwannomatosis, with adulthood benign tumors involving cranial and peripheral nerves. The mechanisms by which SMARCB1 germline mutations predispose to rhabdoid tumors versus schwannomas are still unknown. Here, to understand the origin of these two types of SMARCB1-associated tumors, we generated different tissue- and developmental stage-specific conditional knockout mice carrying Smarcb1 and/or Nf2 deletion. Smarcb1 loss in early neural crest was necessary to initiate tumorigenesis in the cranial nerves and meninges with typical histological features and molecular profiles of human rhabdoid tumors. By inducing Smarcb1 loss at later developmental stage in the Schwann cell lineage, in addition to biallelic Nf2 gene inactivation, we generated the first mouse model developing schwannomas with the same underlying gene mutations found in schwannomatosis patients.

10 January 2014 in Neuro-Oncology

Journal Article

mTORC1 inhibition delays growth of neurofibromatosis type 2 schwannoma

Marco Giovannini

Nicolas-Xavier Bonne

Jeremie Vitte

Fabrice Chareyre

Karo Tanaka

Michel Kalamarides

Neurofibromatosis type 2 (NF2) is a rare autosomal dominant genetic disorder, resulting in a variety of neural tumors, with bilateral vestibular schwannomas as the most frequent manifestation. Recently, merlin, the NF2 tumor suppressor, has been identified as a novel negative regulator of mammalian target of rapamycin complex 1 (mTORC1); functional loss of merlin was shown to result in elevated mTORC1 signaling in NF2-related tumors. Thus, mTORC1 pathway inhibition may be a useful targeted therapeutic approach. We studied in vitro cell models, cohorts of mice allografted with Nf2^−/− Schwann cells, and a genetically modified mouse model of NF2 schwannoma in order to evaluate the efficacy of the proposed targeted therapy for NF2. We found that treatment with the mTORC1 inhibitor rapamycin reduced the severity of NF2-related Schwann cell tumorigenesis without significant toxicity. Consistent with these results, in an NF2 patient with growing vestibular schwannomas, the rapalog sirolimus induced tumor growth arrest. Taken together, these results constitute definitive evidence that justifies proceeding with clinical trials using mTORC1-targeted agents in selected patients with NF2 and in patients with NF2-related sporadic tumors.

About Me

Fascinated by the complexity of human inherited diseases, Dr. Vitte has been working for more than 15 years with mouse models and recently with pig models to understand the molecular and cellular biology involved in these conditions. His interests for neurosciences and oncology lead him to pursue research projects focused on neurodegenerative diseases and on tumor predisposition syndromes of the nervous system. Dr. Vitte is passionate about creating the best animal and cellular models related to a human condition in order to better understand the disease mechanisms and test new therapeutics. Dr. Vitte completed his Ph.D. degree in Cellular and Molecular Biology at Université d’Evry-Val d’Essonne, France in 2007. His research focused on cellular and mouse models of Spinal Muscular Atrophy. During a postdoctoral position in the Neurology and Experimental Therapeutics laboratory at Hopital Pitié-Salpétrière, INSERM, France, he worked on human pathology and mouse models of Parkinson’s disease. In 2009, Dr. Vitte moved to Los Angeles, CA to conduct neuro-oncology research in the Neural Tumor Research laboratory directed by Dr. Marco Giovannini at the House Research Institute and now in the Department of Head and Neck Surgery at the University of California, Los Angeles.His research interests focus on developing and characterizing cellular and animal models, as well as performing pre-clinical research and drug testing for tumor predisposition syndromes.

Dr. Jeremie Vitte

Dr. Jeremie Vitte

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