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201.O1.6 Biology of Severe Congenital Neutropenia and Neutrophil Migration

Monday, December 8, 2008: 11:00 AM-12:30 PM
Yerba Buena Ballroom Salon 7 (San Francisco Marriott)
Moderators:
Daniel C. Link, MD and Masao Kobayashi, MD, PhD
11:00 AM
Deregulation of MicroRNA Underlies Severe Congenital Neutropenia Pathogenesis

Chinavenmeni S. Velu, PhD1, Avinash M Baktula, MS1*, Tristan Bourdeau, MS2* and H. Leighton Grimes, PhD2

1Immunobiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH
2Cincinnati Children's Hospital Medical Center, Cincinnati, OH

11:15 AM
Induction of the Unfolded Protein Response but Normal Basal Granulopoiesis in Mice Expressing G192X ELA2

Mark Murakami*, Jill Woloszynek*, Jun Xia*, Fulu Liu* and Daniel Link, MD

Washington University School of Medicine, St. Louis, MO

11:30 AM
The in Vivo Response of Patients Suffering from Severe Congenital Neutropenia (CN) to G-CSF Is Due to “Emergency” Granulopoiesis Induced by C/EBPß

Lan Dan*, Basant Kumar Thakur*, Julia Skokowa* and Karl Welte

Department of Pediatric Hematology/Oncology, Hannover Medical School, Hannover, Germany

11:45 AM
The Association Between STAT5a Activation, LEF-1 Down Regulation and Leukemic Transformation in Patients with Severe Congenital Neutropenia

Kshama Gupta*, Julia Skokowa* and Karl Welte

Department of Pediatric Hematology/Oncology, Hannover Medical School, Hannover, Germany

12:00 PM
Rho GTPase CDC42 Maintains Neutrophil Polarity Axis during Directed Migration by Regulating CD11b Integrin Signals through Microtubules

Kathleen Szczur*, Yi Zheng* and Marie-Dominique Filippi

Experimental Hematology, Cincinnati Children's Research Foundation, Cincinnati, OH

12:15 PM
Erythrocyte Scaffolding Protein p55 Functions as An Essential Regulator of Neutrophil Polarity

Brendan J. Quinn* and Athar H. Chishti, PhD

College of Medicine, University of Illinois at Chicago, Chicago, IL

*signifies non-member of ASH