SlideShare a Scribd company logo
 
Lymphocyte development McCormack & Rabbitts ,  N Engl J Med 350, 2004, 913-922 GK 2009   ● X-SCID: ●  defective   c -receptor ● on X-chromosome
Known forms of SCID A. Fischer, Stockholm 2005 GK 2009   THYMUS CLP NK CD8 B HSC Myeloid  compartment CD4 Haemotopoietic stem cell Common lymphocyte precursor
Known forms of SCID A. Fischer, Stockholm 2005 GK 2009   THYMUS CLP NK CD8 B HSC Myeloid  compartment CD4 Haemotopoietic stem cell Common lymphocyte precursor 1. Prevention of cell apoptosis DNA replication (purine metabolism) ADA
Known forms of SCID A. Fischer, Stockholm 2005 GK 2009   THYMUS CLP NK CD8 B HSC Myeloid  compartment CD4 Haemotopoietic stem cell Common lymphocyte precursor 2.   c   cytokine-dependent signals      c ,  JAK-3, IL7Ra 1. Prevention of cell apoptosis DNA replication (purine metabolism) ADA
Known forms of SCID A. Fischer, Stockholm 2005 GK 2009   THYMUS CLP NK CD8 B HSC Myeloid  compartment CD4 Haemotopoietic stem cell Common lymphocyte precursor 2.   c   cytokine-dependent signals      c ,  JAK-3, IL7Ra 3. V(D)J recombination Rag-1/-2, Artemis 1. Prevention of cell apoptosis DNA replication (purine metabolism) ADA 4. Pre TCR/TCR signalling) CD45, CD3  , 
Gene therapy: X-SCID  ,[object Object],[object Object],GK 2009   Alain Fischer SCID-patiënts Maria Cavazzana-Calver
Gamma-chain CD3 complex Hacein-Bey-Abina et al., New England J Med 346, 2002, 1185 ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],GK 2009   From: www.med.sc.edu:85/bowers/mhc.htm
CD’s Hacein-Bey-Abina et al NEJM 346, 2002, 1185 ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],GK 2009   CD=cluster of differentiation, marker protein
CD’s Hacein-Bey-Abina et al NEJM 346, 2002, 1185 ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],GK 2009   CD=cluster of differentiation, marker protein
Ex vivo gene therapy From: Sci Am 276-6, 1997, 81 ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],GK 2009
Characteristics of patients Hacein-Bey-Abina et al NEJM 346, 2002, 1185 GK 2009
Numbers of T- and NK- cells after gene transfer  Hacein-Bey-Abina et al NEJM 346, 2002, 1185 GK 2009
Numbers of T- and NK- cells after gene transfer  Hacein-Bey-Abina et al NEJM 346, 2002, 1185 GK 2009
Percentage of transduced cells Hacein-Bey-Abina et al NEJM 346, 2002, 1185 ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],GK 2009
Numbers of naive and memory T-cells  Hacein-Bey-Abina et al NEJM 346, 2002, 1185 GK 2009   ,[object Object],[object Object],[object Object]
Rise of IgA, IgE, and IgG after gene transfer  Hacein-Bey-Abina et al NEJM 346, 2002, 1185 GK 2009
Rise of IgA, IgE, and IgG after gene transfer  Hacein-Bey-Abina et al NEJM 346, 2002, 1185 GK 2009
Can they be vaccinated now? Hacein-Bey-Abina et al NEJM 346, 2002, 1185 GK 2009   Christopher Reid British trial Zachary Hazen DeCruz, French trial
De prijs: Vier kinderen leukemie…  Nature 433, 2005, 561 ,[object Object],[object Object],[object Object],GK 2009   ==============
De prijs: Vier kinderen leukemie…  Nature 433, 2005, 561 ,[object Object],[object Object],[object Object],GK 2009   ============== ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Activation of the T-cell oncogene LMO2   McCormack & Rabbitts N Engl J Med 350, 2004, 913-922 GK 2009   ALL: acute lymphoblastic leukemia ?
Acute leukemia by translocation ,[object Object],[object Object],[object Object],[object Object],GK 2009
Gene   structure of LMO gene McCormack & Rabbitts N Engl J Med 350, 2004, 913-922 ,[object Object],[object Object],GK 2009
Structure and function of LMO2   McCormack & Rabbitts N Engl J Med 350, 2004, 913-922 GK 2009   Two LIM-domains T cell leukemia in mice! LIM domain Normal  erythroid cell Target gene Target gene Block in T-cell differentiation
Random integration? Engelman,  Proc. Natl. Ac. Sci. USA, 102, 2005, 1275-1276 ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],GK 2009   HIV Murine leukemia virus Avian leukosis sarcoma virus
Seven reasons why leukemia might be induced ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],GK 2009
Model of LMO2 function in T-cel leukemia McCormack & Rabbitts N Engl J Med 350, 2004, 913-922 ,[object Object],[object Object],[object Object],GK 2009   Thymus LMO-2-induced differentiation block
Summary: The price…. from: Kaiser, Science 298, 2002, 2113-14 ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],GK 2009   LMO2-gene E1 E2 E3 E4 E5 E6 ============== Exons
Summary: The price…. from: Kaiser, Science 298, 2002, 2113-14 ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],GK 2009   LMO2-gene E1 E2 E3 E4 E5 E6 ============== Exons

More Related Content

What's hot

Association of Angiotensin-converting Enzyme Gene Insertion/ Deletion Polymor...
Association of Angiotensin-converting Enzyme Gene Insertion/ Deletion Polymor...Association of Angiotensin-converting Enzyme Gene Insertion/ Deletion Polymor...
Association of Angiotensin-converting Enzyme Gene Insertion/ Deletion Polymor...
Ibrahim khidir ibrahim osman
 
MInh Project 2
MInh Project 2MInh Project 2
MInh Project 2
Minh Thu Nguyen
 
Step‐by‐Step 
Evolution 
of
 Vertebrate 
Blood
 Coagulation

Step‐by‐Step 
Evolution 
of
 Vertebrate 
Blood
 Coagulation
Step‐by‐Step 
Evolution 
of
 Vertebrate 
Blood
 Coagulation

Step‐by‐Step 
Evolution 
of
 Vertebrate 
Blood
 Coagulation

Madhusudan Katti
 
p21 mechanism slide
p21 mechanism slidep21 mechanism slide
p21 mechanism slide
Wafik El-Deiry, MD PhD FACP
 
Dna damage checkpoints
Dna damage checkpointsDna damage checkpoints
Dna damage checkpoints
Raviz Prathyusha
 
Bethea, John
Bethea, JohnBethea, John
7 immunology-csbrp
7 immunology-csbrp7 immunology-csbrp
7 immunology-csbrp
Prasad CSBR
 
Nf k bnew
Nf k bnewNf k bnew
Nf k bnew
eman youssif
 
Duke neuro 1.10
Duke neuro 1.10Duke neuro 1.10
Duke neuro 1.10
guest05cd529a
 
Restriction enzymes (mc qs)
Restriction enzymes (mc qs)Restriction enzymes (mc qs)
Restriction enzymes (mc qs)
Harshraj Shinde
 
PNS 0502002625
PNS 0502002625PNS 0502002625
PNS 0502002625
Pingtao Tang
 
Clle cycle mostafa
Clle cycle mostafaClle cycle mostafa
Clle cycle mostafa
Mostafa Askar
 
J. Biol. Chem.-2008-Lin-15003-14
J. Biol. Chem.-2008-Lin-15003-14J. Biol. Chem.-2008-Lin-15003-14
J. Biol. Chem.-2008-Lin-15003-14
Da-Wei Lin
 
Mucosal associated
Mucosal associatedMucosal associated
Mucosal associated
Smawi GH
 
Cell replication
Cell replicationCell replication
Cell replication
Genovea
 
Restriction enzyme
Restriction enzymeRestriction enzyme
Restriction enzyme
Muhammad Luthfan
 
Il 17 signaling pathway a new therapeutic target
Il 17 signaling pathway a new therapeutic targetIl 17 signaling pathway a new therapeutic target
Il 17 signaling pathway a new therapeutic target
safoora pordel
 
My oral presentation at the Maize Genetics Conference, 2008, Washington DC
My oral presentation at the Maize Genetics Conference, 2008, Washington DCMy oral presentation at the Maize Genetics Conference, 2008, Washington DC
My oral presentation at the Maize Genetics Conference, 2008, Washington DC
Mihai Miclăuș
 
Fall project
Fall projectFall project
Fall project
Hameeda Naimi
 
Freiburg Nk2009 Jan Spanholtz
Freiburg Nk2009 Jan SpanholtzFreiburg Nk2009 Jan Spanholtz
Freiburg Nk2009 Jan Spanholtz
JanSpanholtz
 

What's hot (20)

Association of Angiotensin-converting Enzyme Gene Insertion/ Deletion Polymor...
Association of Angiotensin-converting Enzyme Gene Insertion/ Deletion Polymor...Association of Angiotensin-converting Enzyme Gene Insertion/ Deletion Polymor...
Association of Angiotensin-converting Enzyme Gene Insertion/ Deletion Polymor...
 
MInh Project 2
MInh Project 2MInh Project 2
MInh Project 2
 
Step‐by‐Step 
Evolution 
of
 Vertebrate 
Blood
 Coagulation

Step‐by‐Step 
Evolution 
of
 Vertebrate 
Blood
 Coagulation
Step‐by‐Step 
Evolution 
of
 Vertebrate 
Blood
 Coagulation

Step‐by‐Step 
Evolution 
of
 Vertebrate 
Blood
 Coagulation

 
p21 mechanism slide
p21 mechanism slidep21 mechanism slide
p21 mechanism slide
 
Dna damage checkpoints
Dna damage checkpointsDna damage checkpoints
Dna damage checkpoints
 
Bethea, John
Bethea, JohnBethea, John
Bethea, John
 
7 immunology-csbrp
7 immunology-csbrp7 immunology-csbrp
7 immunology-csbrp
 
Nf k bnew
Nf k bnewNf k bnew
Nf k bnew
 
Duke neuro 1.10
Duke neuro 1.10Duke neuro 1.10
Duke neuro 1.10
 
Restriction enzymes (mc qs)
Restriction enzymes (mc qs)Restriction enzymes (mc qs)
Restriction enzymes (mc qs)
 
PNS 0502002625
PNS 0502002625PNS 0502002625
PNS 0502002625
 
Clle cycle mostafa
Clle cycle mostafaClle cycle mostafa
Clle cycle mostafa
 
J. Biol. Chem.-2008-Lin-15003-14
J. Biol. Chem.-2008-Lin-15003-14J. Biol. Chem.-2008-Lin-15003-14
J. Biol. Chem.-2008-Lin-15003-14
 
Mucosal associated
Mucosal associatedMucosal associated
Mucosal associated
 
Cell replication
Cell replicationCell replication
Cell replication
 
Restriction enzyme
Restriction enzymeRestriction enzyme
Restriction enzyme
 
Il 17 signaling pathway a new therapeutic target
Il 17 signaling pathway a new therapeutic targetIl 17 signaling pathway a new therapeutic target
Il 17 signaling pathway a new therapeutic target
 
My oral presentation at the Maize Genetics Conference, 2008, Washington DC
My oral presentation at the Maize Genetics Conference, 2008, Washington DCMy oral presentation at the Maize Genetics Conference, 2008, Washington DC
My oral presentation at the Maize Genetics Conference, 2008, Washington DC
 
Fall project
Fall projectFall project
Fall project
 
Freiburg Nk2009 Jan Spanholtz
Freiburg Nk2009 Jan SpanholtzFreiburg Nk2009 Jan Spanholtz
Freiburg Nk2009 Jan Spanholtz
 

Viewers also liked

M-Learning. Una nueva visión del aprendizaje
M-Learning. Una nueva visión del aprendizajeM-Learning. Una nueva visión del aprendizaje
M-Learning. Una nueva visión del aprendizaje
Isidro Navarro
 
Stem cell personalized medicine 2017 plus
Stem cell personalized medicine 2017 plusStem cell personalized medicine 2017 plus
Stem cell personalized medicine 2017 plus
Avi Dey
 
Stem Cell Technology
Stem Cell TechnologyStem Cell Technology
Biological Presentation On Stem Cells
Biological Presentation On Stem CellsBiological Presentation On Stem Cells
Biological Presentation On Stem Cells
guestce7e377
 
Stem cell
 Stem cell Stem cell
Stem cell
Ajay Patidar
 
Stem cells
Stem cellsStem cells
Stem cells
Stem Cell Malaysia
 
Stem cell therapy
Stem cell therapyStem cell therapy
Stem cell therapy
Jassi Bassanpaul
 

Viewers also liked (7)

M-Learning. Una nueva visión del aprendizaje
M-Learning. Una nueva visión del aprendizajeM-Learning. Una nueva visión del aprendizaje
M-Learning. Una nueva visión del aprendizaje
 
Stem cell personalized medicine 2017 plus
Stem cell personalized medicine 2017 plusStem cell personalized medicine 2017 plus
Stem cell personalized medicine 2017 plus
 
Stem Cell Technology
Stem Cell TechnologyStem Cell Technology
Stem Cell Technology
 
Biological Presentation On Stem Cells
Biological Presentation On Stem CellsBiological Presentation On Stem Cells
Biological Presentation On Stem Cells
 
Stem cell
 Stem cell Stem cell
Stem cell
 
Stem cells
Stem cellsStem cells
Stem cells
 
Stem cell therapy
Stem cell therapyStem cell therapy
Stem cell therapy
 

Similar to GKA deel 1 college 4

Biology of t lymphocytes
Biology of t lymphocytesBiology of t lymphocytes
Prof.dr.moh'd sorour bch
Prof.dr.moh'd sorour bchProf.dr.moh'd sorour bch
Prof.dr.moh'd sorour bch
mohammed4450
 
Biology of T lymphocytes.pdf
Biology of T lymphocytes.pdfBiology of T lymphocytes.pdf
Bellander, Bo Michael
Bellander, Bo MichaelBellander, Bo Michael
Bellander, Bo Michael
National Neurotrauma Symposium
 
Winslow (1).ppt
Winslow (1).pptWinslow (1).ppt
Winslow (1).ppt
JLMaravillas
 
Apoptosis seminar f
Apoptosis seminar fApoptosis seminar f
Apoptosis seminar f
Nilesh Chandra
 
Emt and ecm 2013
Emt and ecm 2013Emt and ecm 2013
Emt and ecm 2013
Elsa von Licy
 
GKA deel 1 college 15
GKA deel 1 college 15GKA deel 1 college 15
GKA deel 1 college 15
Biology, Utrecht University
 
T and b cells
T and b cellsT and b cells
T and b cells
Damion Lawrence
 
Van criekinge next_generation_epigenetic_profling_vvumc_uploaded
Van criekinge next_generation_epigenetic_profling_vvumc_uploadedVan criekinge next_generation_epigenetic_profling_vvumc_uploaded
Van criekinge next_generation_epigenetic_profling_vvumc_uploaded
Prof. Wim Van Criekinge
 
Diseases of White Blood Cells apthology slide
Diseases of White Blood Cells apthology slideDiseases of White Blood Cells apthology slide
Diseases of White Blood Cells apthology slide
snehajain78924
 
Bacteria&cancer biomed dec_2013
Bacteria&cancer biomed dec_2013Bacteria&cancer biomed dec_2013
Bacteria&cancer biomed dec_2013
Riccardo Guidi
 
Ph D Swati Dhar
Ph D Swati DharPh D Swati Dhar
Ph D Swati Dhar
Swati Dhar
 
Antibody-Producing Cell Lines Development
Antibody-Producing Cell Lines DevelopmentAntibody-Producing Cell Lines Development
Antibody-Producing Cell Lines Development
creativebiogene1
 
Van criekinge next_generation_epigenetic_profling_vlille
Van criekinge next_generation_epigenetic_profling_vlilleVan criekinge next_generation_epigenetic_profling_vlille
Van criekinge next_generation_epigenetic_profling_vlille
Prof. Wim Van Criekinge
 
RECEPTORS-Sexl-Moriggl.pdf
RECEPTORS-Sexl-Moriggl.pdfRECEPTORS-Sexl-Moriggl.pdf
RECEPTORS-Sexl-Moriggl.pdf
ChaitanyaBopate
 
Noble, Marc
Noble, MarcNoble, Marc
Oncogene activation
Oncogene  activationOncogene  activation
Oncogene activation
Dr. Ishan Y. Pandya
 
Sequencing the Human TCRβ Repertoire on the Ion S5TM
Sequencing the Human TCRβ Repertoire on the Ion S5TMSequencing the Human TCRβ Repertoire on the Ion S5TM
Sequencing the Human TCRβ Repertoire on the Ion S5TM
Thermo Fisher Scientific
 
Molecular basis of head and neck cancer
Molecular basis of head and neck cancerMolecular basis of head and neck cancer
Molecular basis of head and neck cancer
SREENIVAS KAMATH
 

Similar to GKA deel 1 college 4 (20)

Biology of t lymphocytes
Biology of t lymphocytesBiology of t lymphocytes
Biology of t lymphocytes
 
Prof.dr.moh'd sorour bch
Prof.dr.moh'd sorour bchProf.dr.moh'd sorour bch
Prof.dr.moh'd sorour bch
 
Biology of T lymphocytes.pdf
Biology of T lymphocytes.pdfBiology of T lymphocytes.pdf
Biology of T lymphocytes.pdf
 
Bellander, Bo Michael
Bellander, Bo MichaelBellander, Bo Michael
Bellander, Bo Michael
 
Winslow (1).ppt
Winslow (1).pptWinslow (1).ppt
Winslow (1).ppt
 
Apoptosis seminar f
Apoptosis seminar fApoptosis seminar f
Apoptosis seminar f
 
Emt and ecm 2013
Emt and ecm 2013Emt and ecm 2013
Emt and ecm 2013
 
GKA deel 1 college 15
GKA deel 1 college 15GKA deel 1 college 15
GKA deel 1 college 15
 
T and b cells
T and b cellsT and b cells
T and b cells
 
Van criekinge next_generation_epigenetic_profling_vvumc_uploaded
Van criekinge next_generation_epigenetic_profling_vvumc_uploadedVan criekinge next_generation_epigenetic_profling_vvumc_uploaded
Van criekinge next_generation_epigenetic_profling_vvumc_uploaded
 
Diseases of White Blood Cells apthology slide
Diseases of White Blood Cells apthology slideDiseases of White Blood Cells apthology slide
Diseases of White Blood Cells apthology slide
 
Bacteria&cancer biomed dec_2013
Bacteria&cancer biomed dec_2013Bacteria&cancer biomed dec_2013
Bacteria&cancer biomed dec_2013
 
Ph D Swati Dhar
Ph D Swati DharPh D Swati Dhar
Ph D Swati Dhar
 
Antibody-Producing Cell Lines Development
Antibody-Producing Cell Lines DevelopmentAntibody-Producing Cell Lines Development
Antibody-Producing Cell Lines Development
 
Van criekinge next_generation_epigenetic_profling_vlille
Van criekinge next_generation_epigenetic_profling_vlilleVan criekinge next_generation_epigenetic_profling_vlille
Van criekinge next_generation_epigenetic_profling_vlille
 
RECEPTORS-Sexl-Moriggl.pdf
RECEPTORS-Sexl-Moriggl.pdfRECEPTORS-Sexl-Moriggl.pdf
RECEPTORS-Sexl-Moriggl.pdf
 
Noble, Marc
Noble, MarcNoble, Marc
Noble, Marc
 
Oncogene activation
Oncogene  activationOncogene  activation
Oncogene activation
 
Sequencing the Human TCRβ Repertoire on the Ion S5TM
Sequencing the Human TCRβ Repertoire on the Ion S5TMSequencing the Human TCRβ Repertoire on the Ion S5TM
Sequencing the Human TCRβ Repertoire on the Ion S5TM
 
Molecular basis of head and neck cancer
Molecular basis of head and neck cancerMolecular basis of head and neck cancer
Molecular basis of head and neck cancer
 

More from Biology, Utrecht University

Groeneveld 31032011
Groeneveld 31032011Groeneveld 31032011
Groeneveld 31032011
Biology, Utrecht University
 
Basic concepts
Basic conceptsBasic concepts
Zintuigfysiologie (3) (elektromagnetiche receptoren, zien)
Zintuigfysiologie (3) (elektromagnetiche receptoren, zien)Zintuigfysiologie (3) (elektromagnetiche receptoren, zien)
Zintuigfysiologie (3) (elektromagnetiche receptoren, zien)Biology, Utrecht University
 
Zintuigfysiologie (2) (chemoreceptoren, smaak en geur)
Zintuigfysiologie (2) (chemoreceptoren, smaak en geur)Zintuigfysiologie (2) (chemoreceptoren, smaak en geur)
Zintuigfysiologie (2) (chemoreceptoren, smaak en geur)Biology, Utrecht University
 
Zintuigfysiologie (1) (mechanische receptoren, gehoor)
Zintuigfysiologie (1) (mechanische receptoren, gehoor)Zintuigfysiologie (1) (mechanische receptoren, gehoor)
Zintuigfysiologie (1) (mechanische receptoren, gehoor)Biology, Utrecht University
 
Plantenbiologie college 2 deel 2
Plantenbiologie college 2 deel 2Plantenbiologie college 2 deel 2
Plantenbiologie college 2 deel 2
Biology, Utrecht University
 
Plantenbiologie college 2 deel 1
Plantenbiologie college 2 deel 1Plantenbiologie college 2 deel 1
Plantenbiologie college 2 deel 1
Biology, Utrecht University
 
Plantenbiologie college 1 deel 2
Plantenbiologie college 1 deel 2Plantenbiologie college 1 deel 2
Plantenbiologie college 1 deel 2
Biology, Utrecht University
 

More from Biology, Utrecht University (20)

Groeneveld 31032011
Groeneveld 31032011Groeneveld 31032011
Groeneveld 31032011
 
Basic concepts
Basic conceptsBasic concepts
Basic concepts
 
Enzymen regulatie
Enzymen regulatieEnzymen regulatie
Enzymen regulatie
 
Enzymen 5e deel
Enzymen 5e deelEnzymen 5e deel
Enzymen 5e deel
 
Enzymen 4e deel
Enzymen 4e deelEnzymen 4e deel
Enzymen 4e deel
 
Enzymen 3e deel
Enzymen 3e deelEnzymen 3e deel
Enzymen 3e deel
 
Enzymen 2e deel
Enzymen 2e deelEnzymen 2e deel
Enzymen 2e deel
 
Enzymen 1e deel
Enzymen 1e deelEnzymen 1e deel
Enzymen 1e deel
 
Gedragsbiologie (2)
Gedragsbiologie (2)Gedragsbiologie (2)
Gedragsbiologie (2)
 
Gedragsbiologie (1)
Gedragsbiologie (1)Gedragsbiologie (1)
Gedragsbiologie (1)
 
Gedragsbiologie (3)
Gedragsbiologie (3)Gedragsbiologie (3)
Gedragsbiologie (3)
 
Gedragsbiologie (4)
Gedragsbiologie (4)Gedragsbiologie (4)
Gedragsbiologie (4)
 
Zintuigfysiologie (3) (elektromagnetiche receptoren, zien)
Zintuigfysiologie (3) (elektromagnetiche receptoren, zien)Zintuigfysiologie (3) (elektromagnetiche receptoren, zien)
Zintuigfysiologie (3) (elektromagnetiche receptoren, zien)
 
Zintuigfysiologie (2) (chemoreceptoren, smaak en geur)
Zintuigfysiologie (2) (chemoreceptoren, smaak en geur)Zintuigfysiologie (2) (chemoreceptoren, smaak en geur)
Zintuigfysiologie (2) (chemoreceptoren, smaak en geur)
 
Zintuigfysiologie (1) (mechanische receptoren, gehoor)
Zintuigfysiologie (1) (mechanische receptoren, gehoor)Zintuigfysiologie (1) (mechanische receptoren, gehoor)
Zintuigfysiologie (1) (mechanische receptoren, gehoor)
 
Ethiek en dierproeven voor onderwijs
Ethiek en dierproeven voor onderwijsEthiek en dierproeven voor onderwijs
Ethiek en dierproeven voor onderwijs
 
Zenuwstelsel
ZenuwstelselZenuwstelsel
Zenuwstelsel
 
Plantenbiologie college 2 deel 2
Plantenbiologie college 2 deel 2Plantenbiologie college 2 deel 2
Plantenbiologie college 2 deel 2
 
Plantenbiologie college 2 deel 1
Plantenbiologie college 2 deel 1Plantenbiologie college 2 deel 1
Plantenbiologie college 2 deel 1
 
Plantenbiologie college 1 deel 2
Plantenbiologie college 1 deel 2Plantenbiologie college 1 deel 2
Plantenbiologie college 1 deel 2
 

GKA deel 1 college 4

  • 1.  
  • 2. Lymphocyte development McCormack & Rabbitts , N Engl J Med 350, 2004, 913-922 GK 2009 ● X-SCID: ● defective  c -receptor ● on X-chromosome
  • 3. Known forms of SCID A. Fischer, Stockholm 2005 GK 2009 THYMUS CLP NK CD8 B HSC Myeloid compartment CD4 Haemotopoietic stem cell Common lymphocyte precursor
  • 4. Known forms of SCID A. Fischer, Stockholm 2005 GK 2009 THYMUS CLP NK CD8 B HSC Myeloid compartment CD4 Haemotopoietic stem cell Common lymphocyte precursor 1. Prevention of cell apoptosis DNA replication (purine metabolism) ADA
  • 5. Known forms of SCID A. Fischer, Stockholm 2005 GK 2009 THYMUS CLP NK CD8 B HSC Myeloid compartment CD4 Haemotopoietic stem cell Common lymphocyte precursor 2.  c cytokine-dependent signals  c , JAK-3, IL7Ra 1. Prevention of cell apoptosis DNA replication (purine metabolism) ADA
  • 6. Known forms of SCID A. Fischer, Stockholm 2005 GK 2009 THYMUS CLP NK CD8 B HSC Myeloid compartment CD4 Haemotopoietic stem cell Common lymphocyte precursor 2.  c cytokine-dependent signals  c , JAK-3, IL7Ra 3. V(D)J recombination Rag-1/-2, Artemis 1. Prevention of cell apoptosis DNA replication (purine metabolism) ADA 4. Pre TCR/TCR signalling) CD45, CD3  , 
  • 7.
  • 8.
  • 9.
  • 10.
  • 11.
  • 12. Characteristics of patients Hacein-Bey-Abina et al NEJM 346, 2002, 1185 GK 2009
  • 13. Numbers of T- and NK- cells after gene transfer Hacein-Bey-Abina et al NEJM 346, 2002, 1185 GK 2009
  • 14. Numbers of T- and NK- cells after gene transfer Hacein-Bey-Abina et al NEJM 346, 2002, 1185 GK 2009
  • 15.
  • 16.
  • 17. Rise of IgA, IgE, and IgG after gene transfer Hacein-Bey-Abina et al NEJM 346, 2002, 1185 GK 2009
  • 18. Rise of IgA, IgE, and IgG after gene transfer Hacein-Bey-Abina et al NEJM 346, 2002, 1185 GK 2009
  • 19. Can they be vaccinated now? Hacein-Bey-Abina et al NEJM 346, 2002, 1185 GK 2009 Christopher Reid British trial Zachary Hazen DeCruz, French trial
  • 20.
  • 21.
  • 22. Activation of the T-cell oncogene LMO2 McCormack & Rabbitts N Engl J Med 350, 2004, 913-922 GK 2009 ALL: acute lymphoblastic leukemia ?
  • 23.
  • 24.
  • 25. Structure and function of LMO2 McCormack & Rabbitts N Engl J Med 350, 2004, 913-922 GK 2009 Two LIM-domains T cell leukemia in mice! LIM domain Normal erythroid cell Target gene Target gene Block in T-cell differentiation
  • 26.
  • 27.
  • 28.
  • 29.
  • 30.

Editor's Notes

  1. GKA 2009 a.a.m.thomas@uu.nl
  2. GKA 2009 a.a.m.thomas@uu.nl