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Paget's disease is a condition marked by abnormal bone remodeling, involving both excessive bone formation and destruction, predominantly in the elderly. Pagetic vertebral ankylosis is a rare manifestation, often associated with Paget's disease, ankylosing spondylitis, or diffuse idiopathic skeletal hyperostosis. This form of acquired vertebral ankylosis is uncommon and occurs in cases with bone-bridging syndesmophytes or osteophytes.

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Influence of Ethnicity and Deprivation on Occurrence of Paget'S Disease in Greater Manchester, UK.

Calcif Tissue Int

November 2024

Centre for Epidemiology Versus Arthritis, Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology Medicine and Health, University of Manchester, Manchester, UK.

There is important variation in the occurrence of Paget's disease in different regions and populations. There are though few data concerning the occurrence of clinically diagnosed disease in black and ethnic minority groups in the United Kingdom (UK). We undertook an anonymised search using an integrated primary and secondary care-based database in Greater Manchester, covering a population of over 3 million people.

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Causes of Musculoskeletal Pain in Paget's Disease of Bone.

Calcif Tissue Int

November 2024

Centre for Genomics and Experimental Medicine, Institute of Genetics and Cancer, University of Edinburgh, Western General Hospital, Edinburgh, UK.

Article Synopsis
  • Paget's disease of bone (PDB) makes bones grow and change shape in a messy way, which can lead to problems like pain, deafness, and broken bones.
  • A study with 168 people found that about 73% of them had musculoskeletal pain, mainly because of osteoarthritis in joints away from the PDB affected areas.
  • The research showed that pain was more common in older folks and women, and highlighted the need to check each person's pain to treat it correctly.
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Article Synopsis
  • * Researchers analyzed RNA sequencing data to uncover differentially expressed genes (DEGs) and created a protein-protein interaction network to better understand these biomarkers.
  • * Out of 1262 DEGs identified, five key genes were highlighted as significant, with RAC2 being noted as a potential common biomarker for both bone metastasis and Paget's disease of bone.
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Valosin-Containing Protein (VCP)/p97 Oligomerization.

Subcell Biochem

July 2024

Borch Department of Medicinal Chemistry and Molecular Pharmacology, Purdue University, West Lafayette, IN, USA.

Valosin-containing protein (VCP), also known as p97, is an evolutionarily conserved AAA+ ATPase essential for cellular homeostasis. Cooperating with different sets of cofactors, VCP is involved in multiple cellular processes through either the ubiquitin-proteasome system (UPS) or the autophagy/lysosomal route. Pathogenic mutations frequently found at the interface between the NTD domain and D1 ATPase domain have been shown to cause malfunction of VCP, leading to degenerative disorders including the inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia (IBMPFD), amyotrophic lateral sclerosis (ALS), and cancers.

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