Stem Cells: June 2006 Archives

Boost the levels of a gene called NANOG inside a embryonic stem cell, fuse it with a brain cell and the brain cell converts into a stem cell.

The researchers believe that Nanog, alongside other genes, kicks into action a cascade of complex biological machinery that forces cells back into their simplest state, before they have gone down the path of becoming one of the 200 cell types found in the body.

Read the article via The Guardian here.

From the Pediatrics Journal, published today:

Successful Allogeneic Hemopoietic Stem Cell Transplantation in a Child Who Had Anhidrotic Ectodermal Dysplasia With Immunodeficiency
Sophie Dupuis-Girod, MDa,b, Caterina Cancrini, MD, PhDc, Françoise Le Deist , MD, PhDd,1, Paolo Palma, MDc, Christine Bodemer, MDe, Anne Puel, PhDf, Susanna Livadiotti, MDc, Capucine Picard, MDa, Xavier Bossuyt, MD, PhDg, Paolo Rossi, MD, PhDc, Alain Fischer, MD, PhDa,h and Jean-Laurent Casanova, MD, PhDa,f

a Unité d'Immunologie et d'Hématologie Pédiatriques
d Centre d'études des Déficits Immunitaires
e Service de Dermatologie
h Développement Normal et Pathologique du Système Immunitaire, Institut National de la Santé et de la Recherche Médicale U429, Hôpital Necker-Enfants Malades, Paris, France
b Service de Génétique, Hospices Civils de Lyon, Hôpital de l'Hotel Dieu, Lyon, France
c Division of Immunology and Infectious Disease, Bambino Gesù Children's Hospital, University of Rome Tor Vergata, Rome, Italy
f Laboratoire de Génétique Humaine des Maladies Infectieuses, Université René Descartes-Institut National de la Santé et de la Recherche Médicale U550, Faculté de Médecine Necker-Enfants Malades, Paris, France
g Laboratory of Experimental Medicine, University Hospital Leuven, Leuven, Belgium

Anhidrotic ectodermal dysplasia with immunodeficiency is associated with multiple infections and a poor clinical outcome. Hypomorphic mutations in nuclear factor {kappa}B essential modulator (NEMO)/I{kappa}B kinase complex and a hypermorphic mutation in inhibitor {alpha} of nuclear factor {kappa}B (I{kappa}B{alpha}) both result in impaired nuclear factor {kappa}B activation and are associated with X-recessive and autosomal-dominant forms of anhidrotic ectodermal dysplasia with immunodeficiency, respectively. Autosomal-dominant anhidrotic ectodermal dysplasia with immunodeficiency is also associated with a severe T-cell phenotype. It is not known whether hematopoietic stem cell transplantation can cure immune deficiency in children with anhidrotic ectodermal dysplasia with immunodeficiency. A boy with autosomal-dominant anhidrotic ectodermal dysplasia with immunodeficiency and a severe T-cell immunodeficiency underwent transplantation at 1 year of age with haploidentical T-cell–depleted bone marrow after myeloablative conditioning. Engraftment occurred, with full hematopoietic chimerism. Seven years after transplantation, clinical outcome is favorable, with normal T-cell development. As expected, the developmental features of the anhidrotic ectodermal dysplasia syndrome have appeared and persisted. This is the first report of successful hematopoietic stem cell transplantation in a child with anhidrotic ectodermal dysplasia with immunodeficiency. Hematopoietic stem cell transplantation is well tolerated and efficiently cures the profound immunodeficiency associated with autosomal-dominant anhidrotic ectodermal dysplasia with immunodeficiency.

Click here 6/6/6 at 6