[Proliferation, differentiation, and gene expression profile of osteoblast of patient with spondyloepiphyseal dysplasia tarda with progressive arthropathy].
OBJECTIVE
To explore the proliferation, differentiation, and gene expression profile of the osteoblasts (OBs) of patient with spondyloepiphyseal dysplasia tarda with progressive arthropathy (SEDT-PA).


METHODS
OBs from the head of femur of a SEDT-PA patient resected during operation were cultured. ELISA was used to examine the bone-specific alkaline phosphatase (BAP), osteocalcin (OC), and osteoprotegrin (OPC) in the lysate of OBs. The proliferation and survival of the OBs were evaluated with MTT method and (3)H-TDR incorporation. Flow cytometry was used to observe the cell cycle. Northern blotting was used to evaluate the mRNA expression of WISP3, a member of the CCN family, mRNA in the OBs. Gene differential expression microarray was used to determine the cDNA expression. Osteoblasts from the head of femur of a patient about the same age with fracture of femur neck was used as control.


RESULTS
The cultured OBs from the SEDT-PA patient showed a higher survival capacity (0.86 +/- 0.04 vs 0.71 +/- 0.10) and more (3)H-TDR incorporation (1363 +/- 350 vs 867 +/- 128). The expressions of OC and OPG were down-regulated to the 1/4.3 and 1/4.69 of the control respectively. There was no significant difference in the expression of BAP. WISP3 was very lowly expressed in the OBs of the SEDT-PA patient. In comparison with the normal control, there were up-regulation of 22 genes, including those coding chemotactic factors and inflammatory factors, and down-regulation of 16 genes in the OBs of the SEDT-PA patient with a synthetic effect of more rapid proliferation of OBs and reduction of synthesis of extracellular matrix, resulting in osteopenia.


CONCLUSIONS
Not significantly different between SEDT-PA patient and normal person, the WISP3 expression may not be a direct factor of SEDT-PA.
Zhou
Hou-de
HD
Institute of Metabolism and Endocrinology, Second Xiangya Hospital of Central South University, Changsha 410011, China.
Huang
Qiu-xia
QX
Xie
Hui
H
Liu
Min
M
Guo
Li-juan
LJ
Yuan
Ling-qing
LQ
Sui
Guo-liang
GL
Zhai
Mu-xu
MX
Peng
Yi-qun
YQ
Gu
Wei
W
Ni
Jiang-dong
JD
Zhang
Xiang-sheng
XS
Liao
Er-yuan
EY
D000469:Alkaline Phosphatase / Q000096:biosynthesis / Q000235:genetics; D055511:CCN Intercellular Signaling Proteins; D002454:Cell Differentiation; D049109:Cell Proliferation; D002478:Cells, Cultured; D020869:Gene Expression Profiling; D006801:Humans; D018969:Insulin-Like Growth Factor Binding Proteins / Q000096:biosynthesis / Q000235:genetics; D009363:Neoplasm Proteins / Q000096:biosynthesis / Q000235:genetics; D010003:Osteoarthritis / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D015675:Osteocalcin / Q000096:biosynthesis / Q000235:genetics; D010009:Osteochondrodysplasias / Q000150:complications* / Q000235:genetics / Q000473:pathology; D010010:Osteoclasts / Q000473:pathology*; D012333:RNA, Messenger / Q000096:biosynthesis / Q000235:genetics
D055511:CCN Intercellular Signaling Proteins; C400081:CCN6 protein, human; D018969:Insulin-Like Growth Factor Binding Proteins; D009363:Neoplasm Proteins; D012333:RNA, Messenger; D015675:Osteocalcin; D000469:Alkaline Phosphatase