AustralieFrV1, PascalFrancis, Corpus, bibRecord, 004D07

Hematological defects in the oc/oc mouse, a model of infantile malignant osteopetrosis

Identifieur interne : 004D07 ( PascalFrancis/Corpus ); précédent : 004D06; suivant : 004D08

Hematological defects in the oc/oc mouse, a model of infantile malignant osteopetrosis

Auteurs : C. Blin-Wakkach ; A. Wakkach ; P. M. Sexton ; N. Rochet ; G. F. Carle

Source :

Leukemia [ 0887-6924 ] ; 2004.

RBID : Pascal:04-0483813

Descripteurs français

Pascal (Inist)
- Tumeur maligne, Modèle animal, Souris, Ostéopétrose, Enfant, Hématopoïèse, Lymphopoïèse, Ostéoclaste, Hématologie.

English descriptors

KwdEn :
- Animal model, Child, Hematology, Hematopoiesis, Lymphopoiesis, Malignant tumor, Mouse, Osteoclast, Osteopetrosis.

Abstract

Infantile malignant osteopetrosis (IMO) is a rare and lethal disease characterized by an absence of bone resorption due to inactive OCLs. Affected patients display an increased bone mass and hematological defects. The osteopetrotic oc/oc mouse displays a bone phenotype similar to the one observed in IMO patients, and the same gene, Tcirg1, is mutated in this model and in the majority of these patients. Therefore, we explored in oc/oc mice the consequences of the perturbed bone microenvironment on hematopoiesis. We show that the myelomonocytic differentiation is increased, leading to an elevated number of OCLs and dendritic cells. B lymphopoiesis is blocked at the pro-B stage in the bone marrow of oc/oc mouse, leading to a low mature B-cell number. T-cell activation is also affected, with a reduction of IFNγ secretion by splenic CD4 T cells. These alterations are associated with a low IL-7 expression in bone marrow. All these data indicate that the lack of bone resorption in oc/oc mice has important consequences in both myelopoiesis and lymphopoiesis, leading to a form of immunodeficiency. The oc/oc mouse is therefore an appropriate model to understand the hematological defects described in IMO patients, and to derive new therapeutic strategies.

Notice en format standard (ISO 2709)

Pour connaître la documentation sur le format Inist Standard.

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A14	`01`			`@1 GPM FRE2720, CNRS/UNSA, Faculté de Médecine, av de Valombrose @2 Nice @3 FRA @Z 1 aut. @Z 2 aut. @Z 4 aut. @Z 5 aut.`
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A44				`@0 0000 @1 © 2004 INIST-CNRS. All rights reserved.`
A45				`@0 37 ref.`
A47	`01`	`1`		`@0 04-0483813`
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C01	`01`		`ENG`	@0 Infantile malignant osteopetrosis (IMO) is a rare and lethal disease characterized by an absence of bone resorption due to inactive OCLs. Affected patients display an increased bone mass and hematological defects. The osteopetrotic oc/oc mouse displays a bone phenotype similar to the one observed in IMO patients, and the same gene, Tcirg1, is mutated in this model and in the majority of these patients. Therefore, we explored in oc/oc mice the consequences of the perturbed bone microenvironment on hematopoiesis. We show that the myelomonocytic differentiation is increased, leading to an elevated number of OCLs and dendritic cells. B lymphopoiesis is blocked at the pro-B stage in the bone marrow of oc/oc mouse, leading to a low mature B-cell number. T-cell activation is also affected, with a reduction of IFNγ secretion by splenic CD4 T cells. These alterations are associated with a low IL-7 expression in bone marrow. All these data indicate that the lack of bone resorption in oc/oc mice has important consequences in both myelopoiesis and lymphopoiesis, leading to a form of immunodeficiency. The oc/oc mouse is therefore an appropriate model to understand the hematological defects described in IMO patients, and to derive new therapeutic strategies.
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C03	`08`	`X`	`SPA`	`@0 Osteoclasto @5 09`
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N21				`@1 271`
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Format Inist (serveur)

NO :	PASCAL 04-0483813 INIST
ET :	Hematological defects in the oc/oc mouse, a model of infantile malignant osteopetrosis
AU :	BLIN-WAKKACH (C.); WAKKACH (A.); SEXTON (P. M.); ROCHET (N.); CARLE (G. F.)
AF :	GPM FRE2720, CNRS/UNSA, Faculté de Médecine, av de Valombrose/Nice/France (1 aut., 2 aut., 4 aut., 5 aut.); Howard Florey Institute, The University of Melbourne/Victoria/Australie (3 aut.)
DT :	Publication en série; Niveau analytique
SO :	Leukemia; ISSN 0887-6924; Coden LEUKED; Royaume-Uni; Da. 2004; Vol. 18; No. 9; Pp. 1505-1511; Bibl. 37 ref.
LA :	Anglais
EA :	Infantile malignant osteopetrosis (IMO) is a rare and lethal disease characterized by an absence of bone resorption due to inactive OCLs. Affected patients display an increased bone mass and hematological defects. The osteopetrotic oc/oc mouse displays a bone phenotype similar to the one observed in IMO patients, and the same gene, Tcirg1, is mutated in this model and in the majority of these patients. Therefore, we explored in oc/oc mice the consequences of the perturbed bone microenvironment on hematopoiesis. We show that the myelomonocytic differentiation is increased, leading to an elevated number of OCLs and dendritic cells. B lymphopoiesis is blocked at the pro-B stage in the bone marrow of oc/oc mouse, leading to a low mature B-cell number. T-cell activation is also affected, with a reduction of IFNγ secretion by splenic CD4 T cells. These alterations are associated with a low IL-7 expression in bone marrow. All these data indicate that the lack of bone resorption in oc/oc mice has important consequences in both myelopoiesis and lymphopoiesis, leading to a form of immunodeficiency. The oc/oc mouse is therefore an appropriate model to understand the hematological defects described in IMO patients, and to derive new therapeutic strategies.
CC :	002B19; 002B15H
FD :	Tumeur maligne; Modèle animal; Souris; Ostéopétrose; Enfant; Hématopoïèse; Lymphopoïèse; Ostéoclaste; Hématologie
FG :	Rodentia; Mammalia; Vertebrata; Homme; Maladie héréditaire; Ostéochondrodysplasie; Système ostéoarticulaire pathologie; Os; Système ostéoarticulaire
ED :	Malignant tumor; Animal model; Mouse; Osteopetrosis; Child; Hematopoiesis; Lymphopoiesis; Osteoclast; Hematology
EG :	Rodentia; Mammalia; Vertebrata; Human; Genetic disease; Osteochondrodysplasia; Diseases of the osteoarticular system; Bone; Osteoarticular system
SD :	Tumor maligno; Modelo animal; Ratón; Osteopetrosis; Niño; Hematopoyesis; Linfopoyesis; Osteoclasto; Hematología
LO :	INIST-21129.354000120194110090
ID :	04-0483813

Links to Exploration step

Pascal:04-0483813

Le document en format XML

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<front><div type="abstract" xml:lang="en">Infantile malignant osteopetrosis (IMO) is a rare and lethal disease characterized by an absence of bone resorption due to inactive OCLs. Affected patients display an increased bone mass and hematological defects. The osteopetrotic oc/oc mouse displays a bone phenotype similar to the one observed in IMO patients, and the same gene, Tcirg1, is mutated in this model and in the majority of these patients. Therefore, we explored in oc/oc mice the consequences of the perturbed bone microenvironment on hematopoiesis. We show that the myelomonocytic differentiation is increased, leading to an elevated number of OCLs and dendritic cells. B lymphopoiesis is blocked at the pro-B stage in the bone marrow of oc/oc mouse, leading to a low mature B-cell number. T-cell activation is also affected, with a reduction of IFNγ secretion by splenic CD4 T cells. These alterations are associated with a low IL-7 expression in bone marrow. All these data indicate that the lack of bone resorption in oc/oc mice has important consequences in both myelopoiesis and lymphopoiesis, leading to a form of immunodeficiency. The oc/oc mouse is therefore an appropriate model to understand the hematological defects described in IMO patients, and to derive new therapeutic strategies.</div>
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<server><NO>PASCAL 04-0483813 INIST</NO>
<ET>Hematological defects in the oc/oc mouse, a model of infantile malignant osteopetrosis</ET>
<AU>BLIN-WAKKACH (C.); WAKKACH (A.); SEXTON (P. M.); ROCHET (N.); CARLE (G. F.)</AU>
<AF>GPM FRE2720, CNRS/UNSA, Faculté de Médecine, av de Valombrose/Nice/France (1 aut., 2 aut., 4 aut., 5 aut.); Howard Florey Institute, The University of Melbourne/Victoria/Australie (3 aut.)</AF>
<DT>Publication en série; Niveau analytique</DT>
<SO>Leukemia; ISSN 0887-6924; Coden LEUKED; Royaume-Uni; Da. 2004; Vol. 18; No. 9; Pp. 1505-1511; Bibl. 37 ref.</SO>
<LA>Anglais</LA>
<EA>Infantile malignant osteopetrosis (IMO) is a rare and lethal disease characterized by an absence of bone resorption due to inactive OCLs. Affected patients display an increased bone mass and hematological defects. The osteopetrotic oc/oc mouse displays a bone phenotype similar to the one observed in IMO patients, and the same gene, Tcirg1, is mutated in this model and in the majority of these patients. Therefore, we explored in oc/oc mice the consequences of the perturbed bone microenvironment on hematopoiesis. We show that the myelomonocytic differentiation is increased, leading to an elevated number of OCLs and dendritic cells. B lymphopoiesis is blocked at the pro-B stage in the bone marrow of oc/oc mouse, leading to a low mature B-cell number. T-cell activation is also affected, with a reduction of IFNγ secretion by splenic CD4 T cells. These alterations are associated with a low IL-7 expression in bone marrow. All these data indicate that the lack of bone resorption in oc/oc mice has important consequences in both myelopoiesis and lymphopoiesis, leading to a form of immunodeficiency. The oc/oc mouse is therefore an appropriate model to understand the hematological defects described in IMO patients, and to derive new therapeutic strategies.</EA>
<CC>002B19; 002B15H</CC>
<FD>Tumeur maligne; Modèle animal; Souris; Ostéopétrose; Enfant; Hématopoïèse; Lymphopoïèse; Ostéoclaste; Hématologie</FD>
<FG>Rodentia; Mammalia; Vertebrata; Homme; Maladie héréditaire; Ostéochondrodysplasie; Système ostéoarticulaire pathologie; Os; Système ostéoarticulaire</FG>
<ED>Malignant tumor; Animal model; Mouse; Osteopetrosis; Child; Hematopoiesis; Lymphopoiesis; Osteoclast; Hematology</ED>
<EG>Rodentia; Mammalia; Vertebrata; Human; Genetic disease; Osteochondrodysplasia; Diseases of the osteoarticular system; Bone; Osteoarticular system</EG>
<SD>Tumor maligno; Modelo animal; Ratón; Osteopetrosis; Niño; Hematopoyesis; Linfopoyesis; Osteoclasto; Hematología</SD>
<LO>INIST-21129.354000120194110090</LO>
<ID>04-0483813</ID>
</server>
</inist>
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Hematological defects in the oc/oc mouse, a model of infantile malignant osteopetrosis

Hematological defects in the oc/oc mouse, a model of infantile malignant osteopetrosis

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