Phenotypical and functional analysis of B lymphocytes of two siblings with combined immunodeficiency and defective expression of major histocompatibility complex (MHC) class II antigens on mononuclear cells

1987 ◽  
Vol 7 (2) ◽  
pp. 98-106 ◽  
Author(s):  
Ger T. Rijkers ◽  
John J. Roord ◽  
Frits Koning ◽  
Wietse Kuis ◽  
Ben J. M. Zegers
Keyword(s):  
Functional Analysis ◽  
Major Histocompatibility Complex ◽  
B Lymphocytes ◽  
Mhc Class Ii ◽  
Mononuclear Cells ◽  
Class Ii ◽  
Combined Immunodeficiency ◽  
Histocompatibility Complex ◽  
Mhc Class Ii Antigens ◽  
Class Ii Antigens

SIGNAL TRANSDUCTION VIA MHC CLASS II ANTIGENS ON B LYMPHOCYTES

1989 ◽  
Vol 16 (4-5) ◽  
pp. 273-281 ◽  
Author(s):  
N. Mooney ◽  
C. Hivroz ◽  
S. Ziai-Talebian ◽  
C. Grillot-Courvalin ◽  
D. Charron
Keyword(s):  
Signal Transduction ◽  
B Lymphocytes ◽  
Mhc Class Ii ◽  
Class Ii ◽  
Mhc Class Ii Antigens ◽  
Class Ii Antigens

scholarly journals Bone marrow transplantation in major histocompatibility complex class II deficiency: a single-center study of 19 patients

Blood ◽  
1995 ◽  
Vol 85 (2) ◽  
pp. 580-587
Author(s):  
C Klein ◽  
M Cavazzana-Calvo ◽  
F Le Deist ◽  
N Jabado ◽  
M Benkerrou ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Major Histocompatibility Complex ◽  
Bone Marrow Transplantation ◽  
Mhc Class Ii ◽  
Marrow Transplantation ◽  
Class Ii ◽  
Combined Immunodeficiency ◽  
Major Histocompatibility ◽  
Histocompatibility Complex ◽  
Mhc Class Ii Deficiency

Major histocompatibility complex (MHC) class II deficiency (bare lymphocyte syndrome) is a rare inborn error of the immune system characterized by impaired antigen presentation and combined immunodeficiency. It causes severe and unremitting infections leading to progressive liver and lung dysfunctions and death during childhood. As in other combined immunodeficiency disorders, bone marrow transplantation (BMT) is considered the treatment of choice for MHC class II deficiency. We analyzed the files of 19 patients who have undergone BMT in our center. Of the 7 patients who underwent HLA- identical BMT, 3 died in the immediate posttransplant period of severe viral infections, whereas the remaining 4 were cured, with recovery of normal immune functions. Of the 12 patients who underwent HLA-haplo- identical BMT, 3 were cured, 1 was improved by partial engraftment, 7 died of infectious complications due to graft failure or rejection, and 1 is still immunodeficient because of engraftment failure. A favorable outcome in the HLA-non-identical BMT group was associated with an age of less than 2 years at the time of transplantation. All the patients with stable long-term engraftment had persistently low CD4 counts after transplantation (105 to 650/microL at last follow up), but no clear susceptibility to opportunistic infections despite persisting MHC class II deficiency on thymic epithelium and other nonhematopoietic cells. We conclude that HLA-identical and -haploidentical BMT can cure MHC class II deficiency, although the success rate of haploidentical BMT is lower than that in other combined immunodeficiency syndromes. HLA- haploidentical BMT should preferably be performed in the first 2 years of life, before the acquisition of chronic virus carriage and sequelae of infections.

scholarly journals Developmental extinction of major histocompatibility complex class II gene expression in plasmocytes is mediated by silencing of the transactivator gene CIITA.

1994 ◽  
Vol 180 (4) ◽  
pp. 1329-1336 ◽  
Author(s):  
P Silacci ◽  
A Mottet ◽  
V Steimle ◽  
W Reith ◽  
B Mach
Keyword(s):  
Gene Expression ◽  
Major Histocompatibility Complex ◽  
B Lymphocytes ◽  
Mhc Class Ii ◽  
Plasma Cells ◽  
Terminal Differentiation ◽  
Class Ii ◽  
Major Histocompatibility ◽  
Histocompatibility Complex ◽  
Mhc Class Ii Genes

Constitutive major histocompatibility complex (MHC) class II gene expression is tightly restricted to antigen presenting cells and is under developmental control. Cells of the B cell lineage acquire the capacity to express MHC class II genes early during ontogeny and lose this property during terminal differentiation into plasma cells. Cell fusion experiments have suggested that the extinction of MHC class II expression in plasma cells is due to a dominant repression, but the underlying mechanisms are not understood. CIITA was recently identified as an MHC class II transactivator that is essential for MHC class II expression in B lymphocytes. We show here that inactivation of MHC class II genes in plasmocytes is associated with silencing of the CIITA gene. Moreover, experimentally induced expression of CIITA in plasmocytes leads to reexpression of MHC class II molecules to the same level as that observed on B lymphocytes. We therefore conclude that the loss of MHC class II expression observed upon terminal differentiation of B lymphocytes into plasmocytes results from silencing of the transactivator gene CIITA.

Activation of genetically major histocompatibility complex (MHC) class II-deficient B lymphocytes

1989 ◽  
Vol 9 (2) ◽  
pp. 125-131 ◽  
Author(s):  
A. Durandy ◽  
M. Mangeney ◽  
C. Griscelli ◽  
M. Forveille ◽  
F. Le Deist ◽  
...  
Keyword(s):  
Major Histocompatibility Complex ◽  
B Lymphocytes ◽  
Mhc Class Ii ◽  
Class Ii ◽  
Major Histocompatibility ◽  
Histocompatibility Complex
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