H
Hubert Nivet
Researcher at François Rabelais University
Publications - 78
Citations - 4808
Hubert Nivet is an academic researcher from François Rabelais University. The author has contributed to research in topics: Transplantation & Kidney transplantation. The author has an hindex of 29, co-authored 77 publications receiving 4424 citations. Previous affiliations of Hubert Nivet include Just Energy.
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Journal ArticleDOI
Mutations in INVS encoding inversin cause nephronophthisis type 2, linking renal cystic disease to the function of primary cilia and left-right axis determination.
Edgar A. Otto,Bernhard Schermer,Tomoko Obara,John F. O'Toole,Karl S. Hiller,Adelheid M. Mueller,Rainer G. Ruf,Julia Hoefele,Frank Beekmann,Daniel Landau,John W. Foreman,Judith A. Goodship,Tom Strachan,Andreas Kispert,Matthias T.F. Wolf,Marie F. Gagnadoux,Hubert Nivet,Corinne Antignac,Gerd Walz,Iain A. Drummond,Thomas Benzing,Friedhelm Hildebrandt +21 more
TL;DR: The interaction and colocalization in cilia of inversin, nephrocystin and β-tubulin connect pathogenetic aspects of NPHP to PKD, to primary cilia function and to left-right axis determination.
Journal ArticleDOI
Anti–Factor H Autoantibodies Associated with Atypical Hemolytic Uremic Syndrome
Marie-Agnès Dragon-Durey,Chantal Loirat,Sylvie Cloarec,Marie-Alice Macher,Jacques Blouin,Hubert Nivet,Laurence Weiss,Wolf H. Fridman,Véronique Frémeaux-Bacchi +8 more
TL;DR: It is supported for the first time that HUS may occur in a context of an autoimmune disease with the development of anti-FH-specific antibody leading to an acquired FH deficiency, which may lead to the design of new approaches of diagnosis and treatment in plasma exchanges or immunosuppressive therapies.
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The ciliary gene RPGRIP1L is mutated in cerebello-oculo-renal syndrome (Joubert syndrome type B) and Meckel syndrome
Marion Delous,Lekbir Baala,Rémi Salomon,Christine Laclef,Christine Laclef,Jeanette Vierkotten,Kálmán Tory,Christelle Golzio,Tiphanie Lacoste,Laurianne Besse,Laurianne Besse,Catherine Ozilou,Imane Moutkine,Nathan E. Hellman,Isabelle Anselme,Isabelle Anselme,Flora Silbermann,Christine Vesque,Christine Vesque,Christoph Gerhardt,Eleanor Rattenberry,Matthias T.F. Wolf,Marie Claire Gubler,Jelena Martinovic,Férechté Encha-Razavi,Nathalie Boddaert,Marie Gonzales,Marie Alice Macher,Hubert Nivet,Gérard Champion,Jean Pierre Berthélémé,Patrick Niaudet,Fiona McDonald,Friedhelm Hildebrandt,Colin A. Johnson,Michel Vekemans,Corinne Antignac,Ulrich Rüther,Sylvie Schneider-Maunoury,Sylvie Schneider-Maunoury,Tania Attié-Bitach,Sophie Saunier +41 more
TL;DR: Findings show that mutations in RPGRIP1L can cause the multiorgan phenotypic abnormalities found in CORS or MKS, which therefore represent a continuum of the same underlying disorder.
Journal ArticleDOI
Differential Impact of Complement Mutations on Clinical Characteristics in Atypical Hemolytic Uremic Syndrome
Anne-Laure Sellier-Leclerc,Véronique Frémeaux-Bacchi,Marie-Agnès Dragon-Durey,Marie-Alice Macher,Patrick Niaudet,Geneviève Guest,Bernard Boudailliez,François Bouissou,Georges Deschênes,Sophie Gie,Michel Tsimaratos,Michel Fischbach,Denis Morin,Hubert Nivet,Corinne Alberti,Chantal Loirat +15 more
TL;DR: Outcome of HUS in patients with CFH mutation is catastrophic, and posttransplantation outcome is poor in all groups except for the MCP mutation group, and new therapies are urgently needed.
Journal ArticleDOI
Rituximab treatment for severe steroid- or cyclosporine-dependent nephrotic syndrome: a multicentric series of 22 cases.
Vincent Guigonis,Aymeric Dallocchio,Véronique Baudouin,Maud Dehennault,Caroline Hachon-Le Camus,Mickael Afanetti,Jaap W. Groothoff,Brigitte Llanas,Patrick Niaudet,Hubert Nivet,Natacha Raynaud,Sophie Taque,Pierre Ronco,François Bouissou +13 more
TL;DR: RTX was effective in all patients when administered during a proteinuria-free period in association with other IS agents, and when relapses occurred, they were always associated with an increase in CD19 cell count.