Rebecca Buckley
Overview:
The overall emphasis of Dr. Buckley's research is in human T,B and NK cell development and in aberrations in their development and regulation. The work involves three particular areas of investigation: 1) the cellular and molecular bases of genetically-determined human immunodeficiency diseases, 2) the use of bone marrow stem cells to cure genetically-determined immunodeficiency diseases, and 3) the use of human SCID bone marrow stem cell chimeras to study human thymic education, T and B cell ontogeny, tolerance induction and MHC restriction mechanisms. Methodology includes monoclonal antibody (mAb) analyses of lymphocyte phenotypes, a variety of T cell and natural killer (NK) cell functional assays, studies of thymic output by T cell receptor recombination excision circle measurement, studies of T cell diversity by spectratyping, studies of T cell longevity by telomere analysis and assessment of B cell differentiation and function. A unique resource available for her studies is the largest population of patients with genetically-determined immunodeficiency diseases in the U.S., which includes the largest population in the world of longterm SCID chimeras treated at a single center, some of whom have been studied and followed for more than 37 years. The administration of rigorously T cell depleted haploidentical bone marrow stem cells to SCID recipients without pre-transplant conditioning or post-transplant use of immunosuppressive drugs to prevent GVHD provides an unmanipulated system for studying human thymic education, T and B cell ontogeny, MHC restriction mechanisms and tolerance induction. Studies to identify mutations in patients with primary immunodeficiency are continuing, particularly in those with SCID.
Positions:
James Buren Sidbury Distinguished Professor Emeritus of Pediatrics, in the School of Medicine
Pediatrics, Allergy and Immunology
School of Medicine
Professor Emeritus of Pediatrics
Pediatrics, Allergy and Immunology
School of Medicine
Member of the Duke Cancer Institute
Duke Cancer Institute
School of Medicine
Education:
M.D. 1958
University of North Carolina - Chapel Hill
Grants:
Safety and Efficacy of Hizentra in Pediatric BMT
Administered By
Pediatrics, Allergy and Immunology
Awarded By
Carolinas HealthCare System
Role
Principal Investigator
Start Date
End Date
Identifying the Causes of Primary immunodeficiency through Next Generation Sequencing
Administered By
Pediatrics, Allergy and Immunology
Awarded By
Baxter Healthcare Corporation
Role
Principal Investigator
Start Date
End Date
Prospective Study of SCID Infants who receive Hematopoietic Cell Therapy
Administered By
Pediatrics, Allergy and Immunology
Awarded By
University of California - San Francisco
Role
Principal Investigator
Start Date
End Date
Identification of Disease-Causing Mutations in SCID Using Exome-Wide Sequencing
Administered By
Pediatrics, Allergy and Immunology
Awarded By
National Institutes of Health
Role
Investigator
Start Date
End Date
Mechanisms of Allogeneic Stem Cell Education in SCID
Administered By
Pediatrics, Allergy and Immunology
Awarded By
National Institutes of Health
Role
Principal Investigator
Start Date
End Date
Publications:
Development of XSCID gene therapy for posttransplant patients with persistent immune defects.
Authors
MLA Citation
Puck, J. M., et al. “Development of XSCID gene therapy for posttransplant patients with persistent immune defects.” Blood Cells Molecules and Diseases, vol. 28, no. 3, 2002, pp. 343–343.
URI
https://scholars.duke.edu/individual/pub1560661
Source
wos-lite
Published In
Blood Cells, Molecules, and Diseases
Volume
28
Published Date
Start Page
343
End Page
343
The Natural History of Children with Severe Combined Immunodeficiency Disease (SCID): The First Fifty Patients of the Primary Immune Deficiency Treatment Consortium (PIDTC) Prospective Study 6901
Authors
Dvorak, CC; Cowan, MJ; Logan, BR; Griffith, L; Puck, J; Notarangelo, LD; Kohn, DB; Xiang, Q; Eapen, M; Shearer, W; Pulsipher, MA; Buckley, R
MLA Citation
Dvorak, Christopher C., et al. “The Natural History of Children with Severe Combined Immunodeficiency Disease (SCID): The First Fifty Patients of the Primary Immune Deficiency Treatment Consortium (PIDTC) Prospective Study 6901.” Biology of Blood and Marrow Transplantation, vol. 19, no. 2, Elsevier BV, 2013, pp. S161–62. Crossref, doi:10.1016/j.bbmt.2012.11.125.
URI
https://scholars.duke.edu/individual/pub1560655
Source
crossref
Published In
Biology of Blood and Marrow Transplantation : Journal of the American Society for Blood and Marrow Transplantation
Volume
19
Published Date
Start Page
S161
End Page
S162
DOI
10.1016/j.bbmt.2012.11.125
Deficiency of janus kinase 3 (Jak3) protein in severe combined immunodeficiency (SCID)
Authors
Roberts, JL; OShea, JJ; Puck, JM; Buckley, RH
MLA Citation
Roberts, J. L., et al. “Deficiency of janus kinase 3 (Jak3) protein in severe combined immunodeficiency (SCID).” Journal of Allergy and Clinical Immunology, vol. 97, no. 1, 1996, pp. 828–828.
URI
https://scholars.duke.edu/individual/pub1560668
Source
wos-lite
Published In
Journal of Allergy and Clinical Immunology
Volume
97
Published Date
Start Page
828
End Page
828
DEVELOPMENT OF T-CELL FUNCTION AFTER POSTNATAL THYMIC TRANSPLANTATION FOR DIGEORGE-SYNDROME
Authors
MARKERT, ML; WARD, FE; KOSTYU, D; BUCKLEY, RH; SCHIFF, SE; BLEESING, JJH; BROOME, CB; UNGERLEIDER, RM; GAYNOR, JW; MAHAFFEY, SM; OLDHAM, KT; WATSON, TJ; MCLAUGHLIN, TM; HAYNES, BF
MLA Citation
MARKERT, M. L., et al. “DEVELOPMENT OF T-CELL FUNCTION AFTER POSTNATAL THYMIC TRANSPLANTATION FOR DIGEORGE-SYNDROME.” Journal of Allergy and Clinical Immunology, vol. 95, no. 1, 1995, pp. 142–142.
URI
https://scholars.duke.edu/individual/pub1560675
Source
wos-lite
Published In
Journal of Allergy and Clinical Immunology
Volume
95
Published Date
Start Page
142
End Page
142
INDUCTION OF HUMAN IGE AND IGG SYNTHESIS BY INVITRO STIMULATION OF BLOOD MONONUCLEAR-CELLS (MNC) WITH RECOMBINANT HUMAN INTERLEUKIN-4 (RHIL-4)
Authors
CLAASSEN, JL; LEVINE, AD; BUCKLEY, RH
MLA Citation
CLAASSEN, J. L., et al. “INDUCTION OF HUMAN IGE AND IGG SYNTHESIS BY INVITRO STIMULATION OF BLOOD MONONUCLEAR-CELLS (MNC) WITH RECOMBINANT HUMAN INTERLEUKIN-4 (RHIL-4).” Journal of Allergy and Clinical Immunology, vol. 83, no. 1, 1989, pp. 298–298.
URI
https://scholars.duke.edu/individual/pub1560688
Source
wos-lite
Published In
Journal of Allergy and Clinical Immunology
Volume
83
Published Date
Start Page
298
End Page
298
Research Areas:
Acute Disease
Adenosine Deaminase
Agammaglobulinemia
Age Factors
Aged
Allergens
Allergy and Immunology
Amino Acid Sequence
Antibodies
Antibodies, Anti-Idiotypic
Antibodies, Viral
Antibody Formation
Antibody Specificity
Antibody-Producing Cells
Antigen-Antibody Reactions
Antigen-Presenting Cells
Antigens, CD34
Antigens, CD40
Antigens, CD45
Antigens, Surface
Apoptosis
Asthma
Ataxia Telangiectasia
Australia
B-Lymphocytes
Blood
Blood Protein Disorders
Bone Marrow Cells
Bone Marrow Transplantation
Brain
CD40 Antigens
CD40 Ligand
CD8-Positive T-Lymphocytes
Candida albicans
Candidiasis
Cause of Death
Cell Division
Cell Line
Cell Line, Transformed
Cell Membrane
Cerebrospinal Fluid
Child, Preschool
Chimera
Chimerism
Chromatography, Ion Exchange
Chromosome Deletion
Chromosomes, Human, Pair 14
Chromosomes, Human, Pair 4
Chronic Disease
Clinical Trial
Cohort Studies
Colon
Common Variable Immunodeficiency
Consanguinity
Cytokines
DNA
DNA Mutational Analysis
DNA Primers
DNA Transposable Elements
Databases, Factual
Dendritic Cells
Deoxyadenosines
DiGeorge Syndrome
Disease Susceptibility
Double-Blind Method
Duodenum
Echovirus 9
Echovirus Infections
Enterovirus B, Human
Esophagitis
Exons
Fatal Outcome
Female
Flow Cytometry
Follow-Up Studies
Frameshift Mutation
Gastroesophageal Reflux
Gene Deletion
Gene Frequency
Gene therapy
Genes, Dominant
Genes, Recessive
Genetic Carrier Screening
Genetic Diseases, X-Linked
Genetic Markers
Genetic Predisposition to Disease
Genetic Therapy
Genotype
Giardiasis
Goats
Graft Survival
Graft vs Host Disease
Granulocytes
Granulomatous Disease, Chronic
Growth
HLA Antigens
Haplotypes
Hematopoietic Stem Cell Transplantation
Hematopoietic Stem Cells
Herpesvirus 4, Human
Heterozygote Detection
Homozygote
Humans
Hypersensitivity
Hypersensitivity, Immediate
IgA Deficiency
Immune Adherence Reaction
Immune Sera
Immune System
Immune System Diseases
Immunity, Maternally-Acquired
Immunization, Passive
Immunocompromised Host
Immunodiffusion
Immunoenzyme Techniques
Immunoglobulin A
Immunoglobulin Class Switching
Immunoglobulin D
Immunoglobulin E
Immunoglobulin G
Immunoglobulin Isotypes
Immunoglobulin M
Immunoglobulin mu-Chains
Immunoglobulins
Immunoglobulins, Intravenous
Immunologic Deficiency Syndromes
Immunologic Memory
Immunologic Techniques
Immunophenotyping
Immunotherapy
Incidence
Infant
Infant, Newborn
Infection
Infection Control
Injections, Intravenous
Interleukin-13
Interleukin-2
Interleukin-4
Intestine, Small
Janus Kinase 3
Kidney Neoplasms
Killer Cells, Natural
Lectins
Leiomyomatosis
Leukocyte Common Antigens
Leukocytes
Leukocytes, Mononuclear
Liver Function Tests
Loss of Heterozygosity
Lymph Nodes
Lymphangiectasis, Intestinal
Lymphatic Diseases
Lymphocyte Activation
Lymphocyte Count
Lymphocytes
Lymphopenia
Lymphoproliferative Disorders
Major Histocompatibility Complex
Male
Maltose
Medical Records
Membrane Glycoproteins
Mice
Microfluidic Analytical Techniques
Middle Aged
Mitogens
Molecular Sequence Data
Monocytes
Multicenter Studies as Topic
Mutagenesis, Site-Directed
Mutation
Myelodysplastic Syndromes
Neonatal Screening
Neutrophils
Nucleoside Deaminases
Pediatrics
Pedigree
Phagocytes
Phenotype
Phytohemagglutinins
Pneumonia, Aspiration
Point Mutation
Polymerase Chain Reaction
Polymorphism, Single Nucleotide
Polymorphism, Single-Stranded Conformational
Practice Guidelines as Topic
Precursor Cell Lymphoblastic Leukemia-Lymphoma
Prevalence
Prospective Studies
Protein-Tyrosine Kinases
RNA
RNA Splicing
RNA, Messenger
Rabbits
Radioimmunoassay
Receptors, Antigen, B-Cell
Receptors, Antigen, T-Cell
Receptors, Cytokine
Receptors, Interleukin
Receptors, Interleukin-2
Receptors, Interleukin-7
Registries
Retreatment
Retrospective Studies
Rhinitis, Allergic, Seasonal
Rosette Formation
S-Adenosylhomocysteine
STAT6 Transcription Factor
Sequence Analysis, DNA
Serotherapy
Severe Combined Immunodeficiency
Sheep
Siblings
Signal Transduction
Skin Tests
Survival Analysis
Survival Rate
T-Lymphocytes
Thymus Gland
Tomography, X-Ray Computed
Trans-Activators
Transduction, Genetic
Transplantation Chimera
Transplantation Conditioning
Transplantation Immunology
Uniparental Disomy
United States
Unrelated Donors
Up-Regulation
Virus Diseases
Volunteers
Vomiting
Wiskott-Aldrich Syndrome
X Chromosome
Young Adult
James Buren Sidbury Distinguished Professor Emeritus of Pediatrics, in the School of Medicine
Contact:
426 Jones Building, Durham, NC 27710
Box 2898 Med Ctr, Durham, NC 27710