Research Topics
Genomes and Genes | Hanna T GazdaSummaryAffiliation: Harvard University Country: USA Publications
| Collaborators
|
Detail Information
Publications
Frameshift mutation in p53 regulator RPL26 is associated with multiple physical abnormalities and a specific pre-ribosomal RNA processing defect in diamond-blackfan anemiaHanna T Gazda
Division of Genetics and Program in Genomics, The Manton Center for Orphan Disease Research, Children s Hospital Boston, 3 BlackfanCircle, Boston, MA 02115, USA
Hum Mutat 33:1037-44. 2012..We also found a deletion in RPL19 and missense mutations in RPL3 and RPL23A, which may be variants of unknown significance. Together with RPL5, RPL11, and RPS7, RPL26 is the fourth RP regulating p53 activity that is linked to DBA...
Defective ribosomal protein gene expression alters transcription, translation, apoptosis, and oncogenic pathways in Diamond-Blackfan anemiaHanna T Gazda
Department of Pediatric Oncology, Dana Farber Cancer Institute, Boston, Massachusetts, USA
Stem Cells 24:2034-44. 2006..Downregulation of c-myb expression, which causes complete failure of fetal liver erythropoiesis in knockout mice, suggests a link between RPS19 mutations and reduced erythropoiesis in DBA...
Ribosomal protein S24 gene is mutated in Diamond-Blackfan anemiaHanna T Gazda
Division of Genetics, Children s Hospital Boston, Boston, MA 02115, USA
Am J Hum Genet 79:1110-8. 2006..This finding strongly suggests that DBA is a disorder of ribosome synthesis and that mutations in other RP or associated genes that lead to disrupted ribosomal biogenesis and/or function may also cause DBA...
Ribosomal protein L5 and L11 mutations are associated with cleft palate and abnormal thumbs in Diamond-Blackfan anemia patientsHanna T Gazda
Division of Genetics and Program in Genomics, The Manton Center for Orphan Disease Research, Children s Hospital Boston, Boston, MA 02115, USA
Am J Hum Genet 83:769-80. 2008....
Ribosomal protein genes RPS10 and RPS26 are commonly mutated in Diamond-Blackfan anemiaLeana Doherty
Division of Genetics and Program in Genomics, The Manton Center for Orphan Disease Research, Children s Hospital Boston, Boston, MA 02115, USA
Am J Hum Genet 86:222-8. 2010....
RNA and protein evidence for haplo-insufficiency in Diamond-Blackfan anaemia patients with RPS19 mutationsHanna T Gazda
Department of Pediatric Oncology, Dana Farber Cancer Institute, Boston, MA 02115, USA
Br J Haematol 127:105-13. 2004..Our data support the notion that, in addition to rare DBA patients with the deletion of one allele, the disease in certain other RPS19 mutant patients is because of RPS19 protein haplo-insufficiency...
Expression profiling reveals altered satellite cell numbers and glycolytic enzyme transcription in nemaline myopathy muscleDespina Sanoudou
Division of Genetics, Children's Hospital, and Harvard Medical School, Boston, MA 02115, USA
Proc Natl Acad Sci U S A 100:4666-71. 2003..This comprehensive study of downstream molecular consequences of NM gene mutations provides insights in the cellular events leading to the NM phenotype...
Recent insights into the pathogenesis of Diamond-Blackfan anaemiaHanna T Gazda
Children's Hospital Boston, Division of Genetics and Program in Genomics, Boston, MA 02115, USA
Br J Haematol 135:149-57. 2006....
Ddx18 is essential for cell-cycle progression in zebrafish hematopoietic cells and is mutated in human AMLElspeth M Payne
Department of Pediatric Oncology, Dana Farber Cancer Institute, Harvard Medical School, Boston, MA, USA
Blood 118:903-15. 2011....
Abnormalities of the large ribosomal subunit protein, Rpl35a, in Diamond-Blackfan anemiaJason E Farrar
Division of Pediatric Oncology, Department of Oncology, Kimmel Comprehensive Cancer Center
Blood 112:1582-92. 2008..The results also establish that haploinsufficiency of large ribosomal subunit proteins contributes to bone marrow failure and potentially cancer predisposition...
Mutation of ribosomal protein RPS24 in Diamond-Blackfan anemia results in a ribosome biogenesis disorderValerie Choesmel
Laboratoire de Biologie Molé culaire Eucaryote, Universite de Toulouse, 31062 Toulouse, France
Hum Mol Genet 17:1253-63. 2008....
