Alain Fischer

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Alain Fischer (born 11 September 1949 in Paris) is a doctor, professor of pediatric immunology and French researcher in biology.

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Biography

Alain Fischer's father had wanted to become a doctor, but had been prevented from doing so by the numerous clauses established against the Jews of Hungary. Alain Fischer says his father was one of the reasons he has made a medical career choice. [1]

Fischer obtained his medical degree in 1979, and worked with Claude Griscelli at the Neckers-Enfants Malade Hospital. He became Professor of Immunology (PU-PH) at the University of Paris Descartes, and then Director of the Inserm unit, "Normal and pathological development of the immune system", in 1991. He was Head of the Pediatric Immunology and Hematology Unit (UIH) at Necker Hospital from 1996 to 2012. [2]

Fischer was elected in November 2002 as a full member of the French Academy of sciences, and was a member of the National Consultative Ethics Committee from 2005 to 2009. In 2007, he was one of the founding members of the Imagine Institute of Genetic Diseases. [3] In 2008, he received the Inserm Grand Prize for his work. [4] On 5 May 2011, Alain Fischer was elected a full member of the French Academy of Medicine. [5]

In July 2011, he joined Martine Aubry's campaign team for the 2012 presidential election, working with Jean Mallot and Brigitte Dormont on the theme "Health and Social Security". [6]

Alain Fischer has held the chair of experimental medicine at the Collège de France since 15 May 2014, the date of his inaugural lecture. [2] He is a member of the Scientific Council of the IRIS Association. [7]

Scientific contribution

Alain Fischer's work has been focused for years on immunodeficiency acquired from birth (such as bubble babies) and curative approaches using gene therapies. With Marina Cavazzana-Calvo and Salima Hacein-Bey, he obtained in 1999, the first clinical successes in the world of gene therapies for about ten bubble children, [8] two of whom unfortunately developed leukaemias after a few months, one of whom had died. The test was stopped urgently in 2002. Research will show that these leukaemias are due to random insertion into a proto-oncogene of the drug gene. [4] [9] The trial is restarted in 2004, according to a modified protocol using better retroviral vectors, and will be stopped again in 2005 due to new complications. However, of the 16 children treated to date, 15 are satisfactorily cured of their acute immune deficiency.

Awards and honours

Related Research Articles

<span class="mw-page-title-main">Severe combined immunodeficiency</span> Genetic disorder leading to severe impairment of the immune system

Severe combined immunodeficiency (SCID), also known as Swiss-type agammaglobulinemia, is a rare genetic disorder characterized by the disturbed development of functional T cells and B cells caused by numerous genetic mutations that result in differing clinical presentations. SCID involves defective antibody response due to either direct involvement with B lymphocytes or through improper B lymphocyte activation due to non-functional T-helper cells. Consequently, both "arms" of the adaptive immune system are impaired due to a defect in one of several possible genes. SCID is the most severe form of primary immunodeficiencies, and there are now at least nine different known genes in which mutations lead to a form of SCID. It is also known as the bubble boy disease and bubble baby disease because its victims are extremely vulnerable to infectious diseases and some of them, such as David Vetter, have become famous for living in a sterile environment. SCID is the result of an immune system so highly compromised that it is considered almost absent.

<span class="mw-page-title-main">Adenosine deaminase deficiency</span> Medical condition

Adenosine deaminase deficiency is a metabolic disorder that causes immunodeficiency. It is caused by mutations in the ADA gene. It accounts for about 10–20% of all cases of autosomal recessive forms of severe combined immunodeficiency (SCID) after excluding disorders related to inbreeding.

<span class="mw-page-title-main">Wiskott–Aldrich syndrome</span> Medical condition

Wiskott–Aldrich syndrome (WAS) is a rare X-linked recessive disease characterized by eczema, thrombocytopenia, immune deficiency, and bloody diarrhea. It is also sometimes called the eczema-thrombocytopenia-immunodeficiency syndrome in keeping with Aldrich's original description in 1954. The WAS-related disorders of X-linked thrombocytopenia (XLT) and X-linked congenital neutropenia (XLN) may present with similar but less severe symptoms and are caused by mutations of the same gene.

Leukocyte adhesion deficiency (LAD) is a rare autosomal recessive disorder characterized by immunodeficiency resulting in recurrent infections. LAD is currently divided into three subtypes: LAD1, LAD2, and the recently described LAD3, also known as LAD-1/variant. In LAD3, the immune defects are supplemented by a Glanzmann thrombasthenia-like bleeding tendency.

Enzyme replacement therapy (ERT) is a medical treatment which replaces an enzyme that is deficient or absent in the body. Usually, this is done by giving the patient an intravenous (IV) infusion of a solution containing the enzyme.

<span class="mw-page-title-main">X-linked severe combined immunodeficiency</span> Medical condition

X-linked severe combined immunodeficiency (X-SCID) is an immunodeficiency disorder in which the body produces very few T cells and NK cells.

<span class="mw-page-title-main">Inserm</span> Biomedical French research institute

The Institut national de la santé et de la recherche médicale is the French National Institute of Health and Medical Research.

Primary immunodeficiencies are disorders in which part of the body's immune system is missing or does not function normally. To be considered a primary immunodeficiency (PID), the immune deficiency must be inborn, not caused by secondary factors such as other disease, drug treatment, or environmental exposure to toxins. Most primary immunodeficiencies are genetic disorders; the majority are diagnosed in children under the age of one, although milder forms may not be recognized until adulthood. While there are over 430 recognized inborn errors of immunity (IEIs) as of 2019, the vast majority of which are PIDs, most are very rare. About 1 in 500 people in the United States are born with a primary immunodeficiency. Immune deficiencies can result in persistent or recurring infections, auto-inflammatory disorders, tumors, and disorders of various organs. There are currently limited treatments available for these conditions; most are specific to a particular type of PID. Research is currently evaluating the use of stem cell transplants (HSCT) and experimental gene therapies as avenues for treatment in limited subsets of PIDs.

<span class="mw-page-title-main">Artemis (protein)</span> Protein-coding gene in the species Homo sapiens

Artemis is a protein that in humans is encoded by the DCLRE1C gene.

The severe combined immunodeficiency (SCID) is a severe immunodeficiency genetic disorder that is characterized by the complete inability of the adaptive immune system to mount, coordinate, and sustain an appropriate immune response, usually due to absent or atypical T and B lymphocytes. In humans, SCID is colloquially known as "bubble boy" disease, as victims may require complete clinical isolation to prevent lethal infection from environmental microbes.

<span class="mw-page-title-main">T-cell surface glycoprotein CD3 epsilon chain</span> Protein-coding gene in the species Homo sapiens

CD3e molecule, epsilon also known as CD3E is a polypeptide which in humans is encoded by the CD3E gene which resides on chromosome 11.

Hans Dieter Ochs, is an immunologist and pediatrician. He is Professor of Pediatrics, Division of Immunology, Department of Pediatrics, University of Washington School of Medicine, Seattle.

<span class="mw-page-title-main">2011 French Socialist Party presidential primary</span>

The 2011 French Socialist Party presidential primary was the first open primary of the French Socialist Party and Radical Party of the Left for selecting their candidate for the 2012 presidential election. The filing deadline for primary nomination papers was fixed at 13 July 2011 and six candidates competed in the first round of the vote. On election day, 9 October 2011, no candidate won 50 percent of the vote, and the two candidates with the most votes contested a runoff election on 16 October 2011. François Hollande ultimately won the primary, defeating Martine Aubry.

Autologous CD34+ enriched cell fraction that contains CD34+ cells transduced with retroviral vector that encodes for the human ADA cDNA sequence, sold under the brand name Strimvelis, is a medication used to treat severe combined immunodeficiency due to adenosine deaminase deficiency (ADA-SCID).

The 2002 French gene therapy trials were experimental gene therapy trials performed on children suffering from severe combined immunodeficiency (SCID). The trials were based in Paris and led by researchers Alain Fischer and Marina Cavazzana-Calvo. Whilst the experiment initially seemed successful, many of the children began showing symptoms of various cancer-like diseases as a result of the gene manipulation. The experiment, and others like it, were subsequently shut down.

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<span class="mw-page-title-main">Marina Cavazzana</span> Italian physician and cellular biologist

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<span class="mw-page-title-main">Laurent Degos</span> French physician

Laurent Degos, Professor of Haematology at the University of Paris, was born on July 9, 1945, in Paris (75008) from Robert Degos (1904-1987) medical doctor, Professor of Dermatology and Monique Lortat Jacob (1916-1999), third of four children, an older brother Jean Denis (1937-2001) Professor of Neurology, Claude François Professor of Neurology (1939-) and a younger sister Bernadette Flamant (1947-). He was married to Françoise Fouchard (hepatologist) on 16 December 1971 with whom he had three children Juliette Barbarin, Cecile Petit-Degos (scenographer), and Vincent Degos and 9 grandchildren. The Degos family is from Mugron (Landes) with several generations of country medical doctors: Jean Baptiste (1797-1859), Alfred (1840-1925) and Louis (1873-1928) his grandfather.

References

  1. ↑ "Alain Fischer, " La thérapie génique prend son envol "". Le Monde. 7 May 2014.
  2. 1 2 Alain Fischer, médecin et professeur en immunologie pédiatrique, est nommé titulaire de la chaire Médecine expérimentale . Leçon inaugurale, le jeudi 15 mai 2014 à 18h00 , communiqué du Collège de France
  3. ↑ "Institut Imagine des maladies génétiques: le vaisseau de verre de Nouvel". L'Express. 26 April 2011.
  4. 1 2 Alain Fischer récompensé par le Grand Prix Inserm [ permanent dead link ] sur le site du Nouvel Observateur le 2 décembre 2008.
  5. ↑ Le Pr Fischer élu à l’Académie de médecine [ permanent dead link ] sur le site de l'AP-HP.
  6. ↑ L'équipe de campagne de Martine Aubry Archived 3 August 2011 at the Wayback Machine sur le site officiel martineaubry.fr
  7. ↑ « Réactualisation du Conseil scientifique d'IRIS », IRIS, 16 février 2015 (lire en ligne, consulté le 28 novembre 2017)
  8. ↑ Gene Therapy of Human Severe Combined Immunodeficiency (SCID)-X1 Disease, Marina Cavazzana-Calvo, Salima Hacein-Bey, Geneviève de Saint Basile, Fabian Gross, Eric Yvon, Patrick Nusbaum, Françoise Selz, Christophe Hue, Stéphanie Certain, Jean-Laurent Casanova, Philippe Bousso, Françoise Le Deist, and Alain Fischer. Science 28 avril 2000 : Vol. 288. nº 5466, p. 669-672.
  9. ↑ LMO2-associated clonal T cell proliferation in two patients after gene therapy for SCID-X1 S. Hacein-Bey-Abina et al. A. Fischer, M. Cavazzana-Calvo. Science 17 octobre 2003 : Vol. 302. nº 5644, p. 415-419.
  10. ↑ "Laureates of the Japan Prize". japanprize.jp. 2015. Archived from the original on 24 February 2015. Retrieved 30 January 2015..
  11. ↑ Décret du 30 décembre 2017 portant élévation aux dignités de grand'croix et de grand Officier ( Legifrance , 31 décembre 2017)
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