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2 June 2015 EMA/COMP/105743/2013 Rev.1 Committee for Orphan Medicinal Products Murine IgM monoclonal antibody binding to alpha beta T-cell receptor for the prevention of graft rejection following solid organ transplantation First publication 26 March 2013 Rev.1: administrative update 2 June 2015 Disclaimer Please note that revisions to the Public Summary of Opinion are purely administrative updates. Therefore, the scientific content of the document reflects the outcome of the Committee for Orphan Medicinal Products (COMP) at the time of designation and is not updated after first publication. On 12 March 2013, orphan designation (EU/3/13/1113) was granted by the European Commission to CTI Clinical Trial and Consulting Services Europe GmbH, Germany, for murine IgM monoclonal antibody binding to alpha beta T-cell receptor for the prevention of graft rejection following solid organ transplantation. What is graft rejection following solid organ transplantation? Graft rejection following solid organ transplantation is a problem that can occur when the recipient s body rejects the transplanted organ. Graft rejection is caused by the patient s immune system (the body s natural defences) recognising the transplanted graft as foreign. This results in inflammation and damage to the organs. Graft rejection following solid organ transplantation is a life-threatening condition because the transplanted organ may fail and because medication is required to supress the patient s immune system, which can result in infections and malignancies. What is the estimated number of at risk of graft rejection following solid organ transplantation? At the time of designation, the number of patients at risk of organ graft rejection following solid organ transplantation was estimated to be not more than 0.9 people in 10,000 per year in the European 30 Churchill Place Canary Wharf London E14 5EU United Kingdom Telephone +44 (0)20 3660 6000 Facsimile +44 (0)20 3660 5555 Send a question via our website www.ema.europa.eu/contact An agency of the European Union European Medicines Agency, 2015. Reproduction is authorised provided the source is acknowledged.

Union (EU). This was equivalent to a total of not more than 46,000 people per year *, and is below the ceiling for orphan designation. This is based on the information provided by the sponsor and the knowledge of the Committee for Orphan Medicinal Products (COMP). What methods of prevention are available? At the time of designation, several medicines to suppress the immune system in order to prevent rejection after transplantation were authorised in the EU. These include the antibodies basiliximab and rabbit anti-human thymocyte immunoglobulin, which were used at time of transplantation. The sponsor has provided sufficient information to show that this medicine might be of significant benefit to patients at risk of graft rejection because early studies suggest it may be more effective than existing treatments. This assumption will have to be confirmed at the time of marketing authorisation in order to maintain the orphan status. How is this medicine expected to work? This medicine is a monoclonal antibody, a type of protein that has been designed to recognise and attach to a receptor called alpha beta T-cell receptor, which is present on the surface of cells of the immune system called alpha beta T-cells which are involved in the immune response following solid organ transplantation. Alpha beta T-cell receptor is responsible for identifying certain structures (antigens) produced by the transplanted organ as foreign, which initiates the immune response. By attaching to the alpha beta T-cell receptor, this medicine is expected to block its activity, making alpha beta T-cells unable to recognise the antigens. This is expected to reduce the immune reaction to the transplanted organ. What is the stage of development of this medicine? The effects of murine IgM monoclonal antibody binding to alpha beta T-cell receptor have been evaluated in experimental models. At the time of submission of the application for orphan designation, clinical trials with the medicine in patients who received a solid organ transplant were ongoing. At the time of submission, this medicine was not authorised anywhere in the EU for the prevention of graft rejection following solid organ transplantation. Orphan designation of the medicine had been granted in the United States of America for the prevention of this condition. In accordance with Regulation (EC) No 141/2000 of 16 December 1999, the COMP adopted a positive opinion on 6 February 2013 recommending the granting of this designation. * Disclaimer: For the purpose of the designation, the number of patients affected by the condition is estimated and assessed on the basis of data from the European Union (EU 28), Norway, Iceland and Liechtenstein. At the time of designation, this represented a population of 512,200,000 (Eurostat 2013). EMA/COMP/105743/2013 Page 2/5

Opinions on orphan medicinal product designations are based on the following three criteria: the seriousness of the condition; the existence of alternative methods of diagnosis, prevention or treatment; either the rarity of the condition (affecting not more than 5 in 10,000 people in the EU) or insufficient returns on investment. Designated orphan medicinal products are products that are still under investigation and are considered for orphan designation on the basis of potential activity. An orphan designation is not a marketing authorisation. As a consequence, demonstration of quality, safety and efficacy is necessary before a product can be granted a marketing authorisation. For more information Sponsor s contact details: CTI Clinical Trial and Consulting Services Europe GmbH Schillerstrasse 1/15 89077 Ulm Germany Tel. +49 731 4000 8411 Fax +49 731 4000 8429 E-mail: info@ctifacts.com For contact details of patients organisations whose activities are targeted at rare diseases see: Orphanet, a database containing information on rare diseases which includes a directory of patients organisations registered in Europe. European Organisation for Rare Diseases (EURORDIS), a non-governmental alliance of patient organisations and individuals active in the field of rare diseases. EMA/COMP/105743/2013 Page 3/5

Translations of the active ingredient and indication in all official EU languages 1, Norwegian and Icelandic Language Active ingredient Indication English Bulgarian Czech Danish Dutch Estonian Finnish French German Greek Hungarian Italian Latvian Lithuanian Maltese Polish Portuguese Murine IgM monoclonal antibody binding to alpha beta T-cell receptor Мише IgM моноклонално антитяло, свързващо се с αβ Т-клетъчен рецептор Myší monoklonální protilátka IgM vážící se na αβ receptor T-buněk Murint monoklonalt IgM-antistof, der binder til αβ T-celle-receptor Murien IgM monoklonaal antilichaam welke bindt aan αβ T-celreceptor T-raku αβ-retseptoriga liituv hiire monokloonne IgM-antikeha Hiiren monoklonaalinen IgM-luokan vasta-aine, joka sitoutuu T-solujen αβ reseptoreihin Anticorps monoclonal IgM d origine murine se liant au récepteur α/β des lymphocytes T Muriner monoklonaler IgM-Antikörper gegen αβ-t-zellrezeptoren Μονοκλονικό IgM αντίσωμα ποντικού με πρόσδεση στον αβ υποδοχέα των Τ- κυττάρων Az αβ T-sejt receptorhoz kötődő egér IgM monoklonális antitest Anticorpo monoclonale murino IgM che si lega al recettore α/β delle cellule T Peļu IgM monoklonālā antiviela, kas saistās ar αβ T-šūnu receptoru Pelės IgM monokloninis antikūnas, susietas su αβ T ląstelės receptoriumi Antikorp monoklonali IgM tal-ġrieden li jintrabat ma riċettur alfa beta taċ-ċelluli T Mysie przeciwciała monoklonalne IgM wiążące się z receptorem alfa-beta komórek T Anticorpo monoclonal IgM murino que se liga ao receptor alpha/beta das células T. Prevention of graft rejection following solid organ transplantation Предотвратяване на отхвърляне на присадката след трансплантация на солиден орган Prevence rejekce štěpu po transplantaci solidního orgánu Forebyggelse af graftafstødning efter organtransplantation Preventie van transplantaatafstoting na soliede orgaantransplantatie Siirdorgani äratõukamise ennetamine pärast soliidorgani siirdamist Siirrännäisen hylkimisreaktion ehkäisy elinsiirron jälkeen Prévention du rejet de greffe suite à la transplantation d'organes solides Prävention einer Abstoßungsreaktion nach Organtransplantation Πρόληψη της απόρρηψης μοσχεύματος μετά την μεταμόσχευση στερεών οργάνων Szervtranszplantáció után a graft kilökődésének megelőzésére prevenzione del rigetto di trapianto in seguito a trapianto di organi solidi Transplantāta atgrūšanas profilaksei pēc orgāna transplantācijas Transplantato atmetimo prevencija po parenchiminio organo transplantacijos Prevenzjoni ta rifjut ta trapjant wara trapjant ta' organu solidu Zapobieganie odrzucaniu przeszczepu po transplantacji narządów litych Prevenção da rejeição de enxertos após transplante de órgãos sólidos 1 At the time of designation EMA/COMP/105743/2013 Page 4/5

Language Active ingredient Indication Romanian Slovak Slovenian Spanish Swedish Norwegian Icelandic Anticorp IgM monoclonal de origine murină cu legare de receptorul αβ al celulei T Myšacia monoklonálna IgM protilátka viažuca sa na αβ receptory T-lymfocytov Mišje monoklonsko protitelo IgM, ki se veže na receptor αβ limfocitov T Anticuerpo monoclonal murino tipo IgM dirigido contra el receptor alfa-beta de las células T Murin IgM monoklonal antikropp bindande till αβ T-cellsreceptor Murint monoklonalt IgM-antistoff som bindes til en αβ T-cellereseptor Músa einstofna IgM mótefni sem binst alfa beta T-frumu viðtaka Prevenirea respingerii grefei după transplantul de organ solid Prevencia odvrhnutia štepu po orgánovej transplantácii Preprečevanje zavrnitve presadka po transplantaciji čvrstih organov Prevención del rechazo de injerto después de trasplante de órgano sólido Förebyggande av transplantatrejektion efter solid organtransplantation Forebygging av transplantat-avstøting etter solid organ-transplantasjon Til að koma í veg fyrir höfnun eftir líffæraígræðslu EMA/COMP/105743/2013 Page 5/5