Improvement of bone mineral density after enzyme replacement therapy in Chinese late-onset Pompe disease patients

Sheng, Bun; Chu, Yim Pui; Wong, Wa Tai; Yau, Eric Kin Cheong; Chen, Sammy Pak Lam; Luk, Wing Hang

doi:10.1186/s13104-017-2681-y

“…The specific effects of protein lysozyme C, glucosidase and protein disulfideisomerase A5 on OP are still unclear. Studies have reported the correlation with low BMD/OP and the late-onset Pompe disease (LOPD) (Papadimas et al, 2011;Sheng et al, 2017). LOPD is a lysosomal storage disease resulted from deficiency of the enzyme acid a-glucosidase, and enzyme replacement therapy (ERT) with alglucosidase alfa is the only specific treatment available (Van der Ploeg et al, 2010).…”

Section: Discussionmentioning

confidence: 99%

“…LOPD is a lysosomal storage disease resulted from deficiency of the enzyme acid a-glucosidase, and enzyme replacement therapy (ERT) with alglucosidase alfa is the only specific treatment available (Van der Ploeg et al, 2010). In a recent research, patients with LOPD showed improvement in BMD after alglucosidase ERT (Sheng et al, 2017). Protein disulfideisomerase A5 belongs to the protein disulfideisomerase (PDI) family.…”

Section: Discussionmentioning

confidence: 99%

Proteomic profiling analysis of postmenopausal osteoporosis and osteopenia identifies potential proteins associated with low bone mineral density

Huang

¹

,

Wang

²

,

Lv

³

et al. 2020

PeerJ

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Postmenopausal osteoporosis (PMOP) is a major global public health concern and older women are more susceptible to experiencing fragility fractures. Our study investigated the associations between circulating proteins with bone mineral density (BMD) in postmenopausal women with or without low BMD (osteoporosis and osteopenia) using a tandem mass tag (TMT) labeling proteomic experiment and parallel reaction monitoring testing. Across all plasma samples, we quantitatively measured 1,092 proteins, and the OP and normal control (NC) samples were differentiated by principal component analysis and a partial least squares-discrimination analysis model based on the protein profiling data. The differentially abundant proteins between the low BMD and NC samples mostly exhibited binding, molecular function regulator, transporter and molecular transducer activity, and were involved in metabolic and cellular processes, stimulus response, biological regulation, immune system processes and so forth. TMT analysis and RRM validation indicated that the expression of protein Lysozyme C (P61626) was negatively related to BMD, while the expression of proteins Glucosidase (A0A024R592) and Protein disulfideisomerase A5 (Q14554) was positively related to BMD values. Collectively, our results suggest that postmenopausal women with low BMD have a different proteomic profile or signature. Protein alterations may play an important role in the pathogenesis of PMOP, and they may act as novel biomarkers and targets of therapeutic agents for this disease.

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“…The specific effects of protein lysozyme C, glucosidase and protein disulfideisomerase A5 on osteoporosis are still unclear. Studies have reported the correlation with low BMD/osteoporosis and the late-onset Pompe disease (LOPD) (Papadimas et al, 2011;Sheng et al, 2017). LOPD is a lysosomal storage disease resulted from deficiency of the enzyme acid αglucosidase, and enzyme replacement therapy (ERT) with alglucosidase alfa is the only specific treatment available (van der Ploeg et al, 2010).…”

Section: Discussionmentioning

confidence: 99%

“…LOPD is a lysosomal storage disease resulted from deficiency of the enzyme acid αglucosidase, and enzyme replacement therapy (ERT) with alglucosidase alfa is the only specific treatment available (van der Ploeg et al, 2010). In a recent research, patients with LOPD showed improvement in BMD after alglucosidase ERT (Sheng et al, 2017). Protein disulfideisomerase A5 belongs to the protein disulfideisomerase (PDI) family.…”

Section: Discussionmentioning

confidence: 99%

Peer Review #3 of "Proteomic profiling analysis of postmenopausal osteoporosis and osteopenia identifies potential proteins associated with low bone mineral density (v0.1)"

2020

0

View full text Add to dashboard Cite

Postmenopausal osteoporosis (PMOP) is a major global public health concern and older women are more susceptible to experiencing fragility fractures. Our study investigated the associations between circulating proteins with bone mineral density (BMD) in postmenopausal women with or without low BMD (osteoporosis and osteopenia) using a tandem mass tag (TMT) labeling proteomic experiment and parallel reaction monitoring (PRM) testing. Across all plasma samples, we quantitatively measured 1,092 proteins, and the OP and normal control (NC) samples were differentiated by principal component analysis and a partial least squares-discrimination analysis model based on the protein profiling data. The differentially abundant proteins between the low BMD and NC samples mostly exhibited binding, molecular function regulator, transporter, and molecular transducer activity, and were involved in metabolic and cellular processes, stimulus response, biological regulation, immune system processes, and so forth. TMT analysis and RRM validation indicated that the expression of protein Lysozyme C (P61626) was negatively related to BMD, while the expression of proteins Glucosidase (A0A024R592) and Protein disulfideisomerase A5 (Q14554) was positively related to BMD values. Collectively, our results suggest that postmenopausal women with low BMD have a different proteomic profile or signature. Protein alterations may play an important role in the pathogenesis of PMOP, and they may act as novel biomarkers and targets of therapeutic agents for this disease.

show abstract

“…The specific effects of protein lysozyme C, glucosidase and protein disulfideisomerase A5 et al, 2010). In a recent research, patients with LOPD showed 348 improvement in BMD after alglucosidase ERT (Sheng et al, 2017). Protein disulfideisomerase 349 A5 belongs to the protein disulfideisomerase (PDI) family.…”

mentioning

confidence: 99%

Peer Review #2 of "Proteomic profiling analysis of postmenopausal osteoporosis and osteopenia identifies potential proteins associated with low bone mineral density (v0.1)"

2020

0

View full text Add to dashboard Cite

Postmenopausal osteoporosis (PMOP) is a major global public health concern and older women are more susceptible to experiencing fragility fractures. Our study investigated the associations between circulating proteins with bone mineral density (BMD) in postmenopausal women with or without low BMD (osteoporosis and osteopenia) using a tandem mass tag (TMT) labeling proteomic experiment and parallel reaction monitoring (PRM) testing. Across all plasma samples, we quantitatively measured 1,092 proteins, and the OP and normal control (NC) samples were differentiated by principal component analysis and a partial least squares-discrimination analysis model based on the protein profiling data. The differentially abundant proteins between the low BMD and NC samples mostly exhibited binding, molecular function regulator, transporter, and molecular transducer activity, and were involved in metabolic and cellular processes, stimulus response, biological regulation, immune system processes, and so forth. TMT analysis and RRM validation indicated that the expression of protein Lysozyme C (P61626) was negatively related to BMD, while the expression of proteins Glucosidase (A0A024R592) andProtein disulfideisomerase A5 (Q14554) was positively related to BMD values. Collectively, our results suggest that postmenopausal women with low BMD have a different proteomic profile or signature. Protein alterations may play an important role in the pathogenesis of PMOP, and they may act as novel biomarkers and targets of therapeutic agents for this disease.PeerJ reviewing PDF | ABSTRACT 14 Postmenopausal osteoporosis (PMOP) is a major global public health concern and older women 15 are more susceptible to experiencing fragility fractures. Our study investigated the associations 16 between circulating proteins with bone mineral density (BMD) in postmenopausal women with 17 or without low BMD (osteoporosis and osteopenia) using a tandem mass tag (TMT) labeling 18 proteomic experiment and parallel reaction monitoring (PRM) testing. Across all plasma samples, 19 we quantitatively measured 1,092 proteins, and the OP and normal control (NC) samples were 20 differentiated by principal component analysis and a partial least squares-discrimination analysis 21 model based on the protein profiling data. The differentially abundant proteins between the low 22 BMD and NC samples mostly exhibited binding, molecular function regulator, transporter, and 23 molecular transducer activity, and were involved in metabolic and cellular processes, stimulus 24 response, biological regulation, immune system processes, and so forth. TMT analysis and RRM 25 validation indicated that the expression of protein Lysozyme C (P61626) was negatively related 26 to BMD, while the expression of proteins Glucosidase (A0A024R592) and Protein 27 disulfideisomerase A5 (Q14554) was positively related to BMD values. Collectively, our results PeerJ reviewing PDF | Manuscript to be reviewed 28 suggest that postmenopausal women with low BMD have a different proteomic profile or 29 sig...

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Improvement of bone mineral density after enzyme replacement therapy in Chinese late-onset Pompe disease patients

Cited by 7 publications

References 19 publications

Proteomic profiling analysis of postmenopausal osteoporosis and osteopenia identifies potential proteins associated with low bone mineral density

Proteomic profiling analysis of postmenopausal osteoporosis and osteopenia identifies potential proteins associated with low bone mineral density

Peer Review #3 of "Proteomic profiling analysis of postmenopausal osteoporosis and osteopenia identifies potential proteins associated with low bone mineral density (v0.1)"

Peer Review #2 of "Proteomic profiling analysis of postmenopausal osteoporosis and osteopenia identifies potential proteins associated with low bone mineral density (v0.1)"

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