In vitro osteoclastogenesis from Gaucher patients' cells correlates with bone mineral density but not with Chitotriosidase

Gaucher disease (GD) is caused by mutations on the gene encoding for the lysosomal enzyme glucocerebrosidase. Type I GD (GD1) patients present anemia, hepatosplenomegaly and bone alterations. In spite of treatment, bone alterations in GD patients persist, including poor bone mineral density (BMD). M...

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Detalles Bibliográficos
Autores principales: Bondar, Constanza María, Mucci, Juan Marcos, Crivaro, Andrea Natalia, Ormazabal, Maximiliano Emanuel, Ceci, Romina, Oliveri, María Beatriz, González, D., Rozenfeld, Paula Adriana
Formato: Articulo Preprint
Lenguaje:Inglés
Publicado: 2017
Materias:
Ciencias Médicas
Bone mineral density
Bone pathology
Chitotriosidase
Gaucher disease
Osteoclasts
Acceso en línea:http://sedici.unlp.edu.ar/handle/10915/99804
https://ri.conicet.gov.ar/11336/47678
Aporte de:
SEDICI (UNLP) de Universidad Nacional de La Plata
id I19-R120-10915-99804
record_format dspace
institution Universidad Nacional de La Plata
institution_str I-19
repository_str R-120
collection SEDICI (UNLP)
language Inglés
topic Ciencias Médicas
Bone mineral density
Bone pathology
Chitotriosidase
Gaucher disease
Osteoclasts
spellingShingle Ciencias Médicas
Bone mineral density
Bone pathology
Chitotriosidase
Gaucher disease
Osteoclasts
Bondar, Constanza María
Mucci, Juan Marcos
Crivaro, Andrea Natalia
Ormazabal, Maximiliano Emanuel
Ceci, Romina
Oliveri, María Beatriz
González, D.
Rozenfeld, Paula Adriana
In vitro osteoclastogenesis from Gaucher patients' cells correlates with bone mineral density but not with Chitotriosidase
topic_facet Ciencias Médicas
Bone mineral density
Bone pathology
Chitotriosidase
Gaucher disease
Osteoclasts
description Gaucher disease (GD) is caused by mutations on the gene encoding for the lysosomal enzyme glucocerebrosidase. Type I GD (GD1) patients present anemia, hepatosplenomegaly and bone alterations. In spite of treatment, bone alterations in GD patients persist, including poor bone mineral density (BMD). Mechanisms leading to bone damage are not completely understood, but previous reports suggest that osteoclasts are involved. Chitotriosidase (CHIT) is the most reliable biomarker used in the follow up of patients, although its correlation with bone status is unknown. The aim of this work was to study the pro-osteoclastogenic potential in patients and to evaluate its correlation with CHIT activity levels and clinical parameters. PBMCs from treated patients and healthy controls were cultured in the presence of M-CSF, and mature osteoclasts were counted. BMD, blood CHIT activity and serum levels of CTX, BAP, and cytokines were evaluated in patients. We found that blood CHIT activity and osteoclast differentiation were significantly increased in patients, but no correlation between them was observed. Interestingly, osteoclast numbers but not CHIT, presented a negative correlation with BMD expressed as Z-score. CTX, BAP and serum cytokines involved in bone remodeling were found altered in GD1 patients. These results show for the first time a correlation between osteoclast differentiation and BMD in GD1 patients, supporting the involvement of osteoclasts in the bone pathology of GD1. Our results also suggest that an altered immune response may play an important role in bone damage.
format Articulo
Preprint
author Bondar, Constanza María
Mucci, Juan Marcos
Crivaro, Andrea Natalia
Ormazabal, Maximiliano Emanuel
Ceci, Romina
Oliveri, María Beatriz
González, D.
Rozenfeld, Paula Adriana
author_facet Bondar, Constanza María
Mucci, Juan Marcos
Crivaro, Andrea Natalia
Ormazabal, Maximiliano Emanuel
Ceci, Romina
Oliveri, María Beatriz
González, D.
Rozenfeld, Paula Adriana
author_sort Bondar, Constanza María
title In vitro osteoclastogenesis from Gaucher patients' cells correlates with bone mineral density but not with Chitotriosidase
title_short In vitro osteoclastogenesis from Gaucher patients' cells correlates with bone mineral density but not with Chitotriosidase
title_full In vitro osteoclastogenesis from Gaucher patients' cells correlates with bone mineral density but not with Chitotriosidase
title_fullStr In vitro osteoclastogenesis from Gaucher patients' cells correlates with bone mineral density but not with Chitotriosidase
title_full_unstemmed In vitro osteoclastogenesis from Gaucher patients' cells correlates with bone mineral density but not with Chitotriosidase
title_sort in vitro osteoclastogenesis from gaucher patients' cells correlates with bone mineral density but not with chitotriosidase
publishDate 2017
url http://sedici.unlp.edu.ar/handle/10915/99804
https://ri.conicet.gov.ar/11336/47678
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