Plasma proteins present in human cortical bone: enrichment of the alpha2HS-glycoprotein. by Triffitt, J

“…The plasma alpha2HS-glycoprotein concentrations of patients with Paget's disease of bone were shown to be substantially lower than the normal range, with significant negative correlation between the alpha2HS-glycoprotein concentration and the plasma alkaline phosphatase activity.…”

Lymphedema and Paget’s Disease: beyond the nipple by Sansin Tuzun, Aslinur Keles, Narmin Ahmadli

“… Lymphedema praecox is a rare lymphatic vascular anomaly diagnosed before the age of 35, presenting with unilateral involvement of the lower extremities. Paget's Disease of Bone (PDB) is also a rare osteometabolic disorder characterized by an accelerated rate of bone remodeling, and diagnosis of the disease is challenging. …”
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Oral Complications in Cancer Patients–Medication-Related Osteonecrosis of the Jaw (MRONJ) by Cesar Augusto Migliorati

“…Today, because of the effect of the medications in the bone remodeling system, patients with several other diseases such as giant cell tumors, rheumatoid arthritis, Paget's disease of bone, fibrous dysplasia, osteogenesis imperfecta, are managed with these medications, significantly increasing the population of individuals at risk for developing MRONJ. …”
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A Fine Balance of Dietary Lipids Improves Pathology of a Murine Model of VCP-Associated Multisystem Proteinopathy. by Katrina J Llewellyn, Naomi Walker, Christopher Nguyen, Baichang Tan, Lbachir BenMohamed, Virginia E Kimonis, Angèle Nalbandian

“…VCP disease, caused by mutations in the VCP gene, are a clinically and genetically heterogeneous group of disorders with manifestations varying from hereditary inclusion body myopathy, Paget's disease of bone, frontotemporal dementia (IBMPFD), and amyotrophic lateral sclerosis (ALS). …”
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Diphosphonates in Paget's disease. by Russell, R, Smith, R, Preston, C, Walton, R, Woods, C

“…The diphosphonate, sodium etidronate (disodium ethane-1-hydroxy-1,1-diphosphonate) (E.H.D.P.), which inhibits the growth and dissolution of calcium phosphate crystals in vitro, was given at doses of 0, 1, 5, 10, or 20 mg. per kg. per day for up to 6 months to forty-seven patients with Paget's disease of bone. There was a dose-related suppression of the raised alkaline phosphatase in plasma and total hydroxyproline in urine, and at 20 mg. per kg. per day nearly half of the patients had normal values at the end of treatment. …”

Plasma proteins present in human cortical bone: Enrichment of the αHS-glycoprotein by Ashton, B, Höhling, H, Triffitt, J

“…The plasma αHS-glycoprotein concentrations of patients with Paget's disease of bone were shown to be substantially lower than the normal range, with significant negative correlation between the αHS-glycoprotein concentration and the plasma alkaline phosphatase activity. © 1976 Springer-Verlag.…”

Clinical disorders of bone resorption. by Russell, G, Mueller, G, Shipman, C, Croucher, P

“…Clinical disorders in which bone resorption is increased are very common and include Paget's disease of bone, osteoporosis, and the bone changes secondary to cancer, such as occur in myeloma and metastases from breast cancer. …”

Permian metabolic bone disease revealed by microCT: Paget's disease-like pathology in vertebrae of an early amniote. by Yara Haridy, Florian Witzmann, Patrick Asbach, Robert R Reisz

“…This supports that the varanopid suffered from a metabolic bone disease similar to Paget's disease of bone as seen in humans today, which is linked to both genetic and viral factors. …”
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Bisphosphonates and osteonecrosis of jaws by Geetha Vijay, Vijay Raghavan

“…Bisphosphonates are used to treat osteoporosis. Paget disease of bone and other metabolic bone diseases, multiple myeloma, and skeletal events associated with metastatic neoplasms- In 2003, the first reports describing osteonecrosis of the jaw (ONJ) in patients receiving bisphosphonates were published. …”
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Woven bone formation and mineralization by rat mesenchymal stromal cells imply increased expression of the intermediate filament desmin by Giusy Di Conza, Fulvio Barbaro, Nicoletta Zini, Nicoletta Zini, Giulia Spaletta, Giulia Remaggi, Lisa Elviri, Salvatore Mosca, Silvio Caravelli, Massimiliano Mosca, Roberto Toni, Roberto Toni, Roberto Toni, Roberto Toni

“…Non-union fractures and endocrine – metabolic bone disorders like fibrous dysplasia and Paget disease of bone might take advantage of this molecular evidence for their early diagnosis and follow-up.…”
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Chronic femoral diaphyseal osteomyelitis with radiographs initially concerning for Paget disease of the bone by Seth A. Reasoner, BA, Juan M. Colazo, BSc, Jonathan Tucci, MD, PhD, Justin Cates, MD, PhD, Kathryn M. Dahir, MD

“…Keywords: Chronic osteomyelitis, Paget disease of bone, Bone infection, Metabolic bone disease…”
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Establishment of a TNFRSF11B knock-out human induced pluripotent stem cell line (KSCBi002-B-2) via CRISPR/Cas9 system by Jujin Jeong, Dahye Lee, Byeong-Cheol Park, Tae-Hoon Lee, Sang-Wook Park

“…This knock-out hiPSCs will provide a chance to study Paget disease of bone 5 (juvenile Paget disease).…”
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Exercise training reverses skeletal muscle atrophy in an experimental model of VCP disease. by Angèle Nalbandian, Christopher Nguyen, Veeral Katheria, Katrina J Llewellyn, Mallikarjun Badadani, Vincent Caiozzo, Virginia E Kimonis

“…Mutations in the valosin containing protein (VCP) gene lead to Inclusion body myopathy associated with Paget's disease of bone and frontotemporal dementia (IBMPFD) and more recently affect 2% of amyotrophic lateral sclerosis (ALS)-diagnosed cases.The present investigation was undertaken to examine the effects of uphill and downhill exercise training on muscle histopathology and the autophagy cascade in an experimental VCP mouse model carrying the R155H mutation. …”
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Roles and mechanisms of optineurin in bone metabolism by Junjie Jin, Ruiqi Huang, Yixing Chang, Xuejie Yi

“…These effects are closely related to the development of bone metabolism disorders, such as Paget's disease of bone, rheumatoid arthritis, and osteoporosis. …”
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Bisphosphonates: an update on mechanisms of action and how these relate to clinical efficacy. by Russell, R, Xia, Z, Dunford, J, Oppermann, U, Kwaasi, A, Hulley, P, Kavanagh, K, Triffitt, J, Lundy, M, Phipps, R, Barnett, B, Coxon, F, Rogers, M, Watts, N, Ebetino, F

“…The bisphosphonates (BPs) are well established as the treatments of choice for disorders of excessive bone resorption, including Paget's disease of bone, myeloma and bone metastases, and osteoporosis. …”

The homozygote VCP(R¹⁵⁵H/R¹⁵⁵H) mouse model exhibits accelerated human VCP-associated disease pathology. by Angèle Nalbandian, Katrina J Llewellyn, Masashi Kitazawa, Hong Z Yin, Mallikarjun Badadani, Negar Khanlou, Robert Edwards, Christopher Nguyen, Jogeshwar Mukherjee, Tahseen Mozaffar, Giles Watts, John Weiss, Virginia E Kimonis

“…Valosin containing protein (VCP) mutations are the cause of hereditary inclusion body myopathy, Paget's disease of bone, frontotemporal dementia (IBMPFD). …”
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Pathogenic VCP/TER94 alleles are dominant actives and contribute to neurodegeneration by altering cellular ATP level in a Drosophila IBMPFD model. by Ya-Chu Chang, Wan-Tzu Hung, Yun-Chin Chang, Henry C Chang, Chia-Lin Wu, Ann-Shyn Chiang, George R Jackson, Tzu-Kang Sang

“…Inclusion body myopathy with Paget's disease of bone and frontotemporal dementia (IBMPFD) is caused by mutations in Valosin-containing protein (VCP), a hexameric AAA ATPase that participates in a variety of cellular processes such as protein degradation, organelle biogenesis, and cell-cycle regulation. …”
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Bisphosphonates: from bench to bedside. by Russell, R

“…In clinical medicine, several BPs are established as the treatments of choice for various diseases of excessive bone resorption, including Paget's disease of bone, myeloma and bone metastases, and osteoporosis. …”

Bisphosphonates: mode of action and pharmacology. by Russell, R

“…Their principal uses are for Paget disease of bone, myeloma, bone metastases, and osteoporosis in adults, but there has been increasing and successful application in pediatric bone diseases, notably osteogenesis imperfecta. …”

A study of intracellular orthophosphate concentration in human muscle and erythrocytes by 31P nuclear magnetic resonance spectroscopy and selective chemical assay. by Bevington, A, Mundy, K, Yates, A, Kanis, J, Russell, R, Taylor, D, Rajagopalan, B, Radda, G

“…In order to study the relationship between extracellular and intracellular concentrations of orthophosphate (Pi), phosphorus nuclear magnetic resonance spectra were recorded, at rest, from the flexor digitorum superficialis muscle of hypophosphataemic patients with vitamin D-resistant rickets, and patients with Paget's disease of bone before and after they had been made hyperphosphataemic by treatment with the drug ethylidene-1-hydroxy-1,1-bisphosphonate. …”