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Bardet-Biedl syndrome (BBS) is a rare and highly heterogeneous autosomal recessive disease caused by ciliary structure abnormality or dysfunction. Here we report a case of a 23-year-old woman who was diagnosed with BBS with a rare BBS10 gene mutation. Literature review was performed with a focus to outline treatment and management plans for patients with this rare and potentially dangerous disease.
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Bone remodeling is an ordered process of coupling of bone resorption and bone formation. Cytokines secreted by different bone cells and membrane molecules expressed by bone cells are involved in regulation of coupling of bone resorption and bone formation. To reveal the mechanism of bone coupling is of great significance in the study and diagnosis of osteoporosis and other metabolic bone diseases. In the present review, we summarize the progression of mechanisms by which cytokines and the related membrane molecules are regulating the coupling of bone resorption and bone formation.
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Objective To investigate the effect and mechnism of preptin on connect tissue growth factor (CTGF) in human osteoblasts. Methods Recombinant human preptin was used to treat primary human osteoblasts, and Western blot was used to detect CTGF protein level. Mitogen-activated protein kinase p38(p38MAPK), extracellular signal-regulated kinase (ERK1/2), c-jun N-terminal Kinase (JNK), and their phosphorylation levels were also detected by Western blot. MAPK inhibitors (PD98059, SP600125, or SB203580)were used to elucidate the mechnism of preptin induced expression of CTGF in human osteoblasts. Results Treatment of human osteoblasts with preptin caused a time and dose-dependent increase in CTGF secretion. Preptin induced activation of ERK, but not p38MAPK or JNK in human osteoblasts. Furhermore, pretreatment of human osteoblasts with the ERK inhibitor PD98059 abolished the preptin-induced CTGF secretion. Conclusion Preptin induces CTGF expression in human osteoblasts by means of ERK/MAPK pathway.
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Objective To investigate the effect of preptin on proliferation and differentiation of human osteoblasts. Methods After human osteoblasts were incubated with 10-10, 10-9, 10-8 , 10-7 mol/L preptin for 24 h,the proliferation of osteoblasts was determined by[3H]thymidine incorporation and alkaline phosphatase (ALP)activity was assayed by spectrophotometric measurement. The phosphorylation levels of c-Jun N-terminal kinase (JNK), p38 mitogen-activated protein kinase ( MAPK), extracellular signal-regulated kinase (ERK) 1/2 were assayed by Western blot. ERK inhibitor PD98059, p38MAPK inhibitor SB203580, and JNK inhibitor SP600125were used for investigating the signal pathway of preptin-stimulated osteoblast proliferation and differentiation.Results Preptin dose-dependently increased human proliferation of osteoblasts and ALP activity with the maximum effect at the concentration of l0-9 mol/L (both P<0.01 ). Preptin stimulated ERK phosphorylation in human osteoblasts, but not p38 MAPK and JNK phosphorylation. PD98059 blocked preptin-sitmulated human osteoblasts proliferation and ALP activity (both P<0.05 ), while SB203580 and SP600125 had no effect. Conclusions Preptin promotes the proliferation and differentiation of human osteoblasts through ERK pathway.
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Pancreatic islet β-cell mass is regulated by β-cell replication, nengenesis, apnptosis and cell size. β-cetl mass in diahetic patients is conspicuously less than that in normal subjects. Induction of β-cell regeneration and inhibition of β-cell apoptosis is the new target of diabetes treatment.
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BACKGROUND: The main role that the osteoprotegedn (OPG) plays in bone tissues is to inhibilate the formation and the activity of osteoclast, while as for the receptor activator of nuclear factor-κB ligand (RANKL), it is to stimulate the differentiation and the activity of osteoclast. Both OPG and RANKL are important for the regulation of osteoclastic function. Recent studies have found the stimulative effects of adiponectin on the proliferation and the differentiation of osteoblast, as well as the coupling between adiponectin and bone metabolism. But effects of adiponectin on osteoclast remain unclear. OBJECTIVE: To investigate the effect of adiponectin on the expression of OPG and RANKL in osteoblast, and to further analyze its effects on the formation of osteoclast. DESIGN, TIME AND SETTING: A contrast observational experiment was conducted in the laboratory of Institute of 2008.MATERIALS: Cancellous bone in anterior superior lilac spine was obtained from adult normal by surgery for cell incubation. Clinical samples were provided by the Xiangya Hospital. METHODS: Human osteoblast was incubated with different doses of adiponcetin (0, 3, 10 and 30 mg/L) for 48 hours, after which OPG and RANKL mRNA and protein expression level was determined. In addition, adiponcetin was added into the co-culture system of osteoblast and peripheral blood monouclear cells for examing its effects on osteoclast formation. MAIN OUTCOME MEASURES: OPG and RANKL mRNA and protein expression in human osteoblast was determined using fluorescent quantitative polymerase chain reaction (PCR) and enzyme linked immunosorbent assay (ELISA). Osteoclast was detected by antitartaric acid acid phosphatase staining. RESULTS: Adiponcetin inhibited osteoblast OPG mRNA and protein expression in a dose-dependent way (P < 0.05). Adiponcetin promoted osteoblast RANKL mRNA and protein expression in a dose-dependent way (P < 0.05). Adiponectin induced the osteoclasts formation in the co-culture system of osteoblast and peripheral blood monouclear cells. CONCLUSION: Adiponectin has the effect of inducing osteoclast formation via stimulating RANKL expression and inhibiting OPG
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This in vitro study demonstrated that taurine supplemented culture medium enhanced alkaline phosphatase (ALP) activity, osteocalcin secretion and mineralized matrix formation. Taurine induced activation of ERKI/2 and osteoblast differentiation, which was blocked by pretreatment of osteoblasts with ERKI/2 inhibitor (PD98059), suggesting taurine stimulated osteoblast differentiation via ERKI/2.