Results 81 to 90 of about 3,792 (182)

Future of fibrodysplasia ossificans progressiva management: A systematic review of investigational therapies

Rheumatology &Autoimmunity, Volume 5, Issue 1, Page 28-36, March 2025.
Fibrodysplasia ossificans progressiva, a rare genetic disorder caused by ACVR1 mutations, leads to soft tissue ossification. Recent advancements, including food and drug administration‐approved palovarotene, show promise despite safety concerns. Therapies like tofacitinib and imatinib demonstrate potential, but larger, controlled trials are essential ...
Muhammad Ikrama, Muhammad Usama, Shifa Israr, Maryam Humayon, Muhammad Umair Javaid, Shafqat Abbas, Muhammad Aliyan Haider   +6 more
wiley   +1 more source

Promiscuous signaling of ligands via mutant ALK2 in fibrodysplasia ossificans progressiva [PDF]

, 2016
Fibrodysplasia ossificans progressiva (FOP) is a rare hereditary disorder characterized by successive heterotopic bone formation, for which at present there is no therapy. Mutations in the bone morphogenetic protein (BMP) type I receptor Activin receptor-
David Jan Jozef de Gorter, Gonzalo Sánchez-Duffhues, Peter ten Dijke
core   +1 more source

ECSIT‐X4 is Required for Preventing Pressure Overload‐Induced Cardiac Hypertrophy via Regulating Mitochondrial STAT3

Advanced Science, Volume 12, Issue 8, February 24, 2025.
ECSIT‐X4 is the predominant cardiac isoform of ECSIT, encoded by the Ecsit‐X4 transcript variant in adult cardiomyocytes. The splicing junction of exon 9 generates the mouse Ecsit‐X4 isoform, resulting in a recognizable C‐terminal sequence. It is shown that ECSIT‐X4 interacts with STAT3, enhancing the activity of complex I, thereby promoting ...
Xia Lu, Tingting Tong, Haoliang Sun, Yi Chen, Yongfeng Shao, Pengxi Shi, Linli Que, Li Liu, Guoqing Zhu, Qi Chen, Chuanfu Li, Jiantao Li, Shuo Yang, Yuehua Li   +13 more
wiley   +1 more source

Fibrodysplasia Ossificans Progressiva (FOP): A Segmental Progeroid Syndrome

Frontiers in Endocrinology, 2020
Segmental progeroid syndromes are commonly represented by genetic conditions which recapitulate aspects of physiological aging by similar, disparate, or unknown mechanisms.
Robert J. Pignolo, Robert J. Pignolo, Robert J. Pignolo, Haitao Wang, Haitao Wang, Haitao Wang, Frederick S. Kaplan, Frederick S. Kaplan, Frederick S. Kaplan   +8 more
doaj   +1 more source

The Hedgehog–GLI1 Pathway Regulates Osteogenic Differentiation of Human Cervical Posterior Longitudinal Ligament Cells by BMP Signalling Pathway

Journal of Cellular and Molecular Medicine, Volume 29, Issue 3, February 2025.
ABSTRACT Cervical ossification of the posterior longitudinal ligament (OPLL) is an ectopic ossification disorder characterised by endochondral ossification. Its aetiology remains to be fully elucidated. This study aimed to clarify its pathogenesis through RNA sequencing of primary cells cultured from patients without cervical OPLL (control, PLL) and ...
Wenbo Xu, Bingbing Ran, Toshimi Aizawa, Wanguo Liu, Jianhui Zhao, Renrui Niu, Zeping Liu, Rui Gu   +7 more
wiley   +1 more source

Enhanced BMP Signaling Alters Human β‐Cell Identity and Function

Advanced Biology, Volume 9, Issue 1, January 2025.
This study evaluates the association between bone morphogenetic protein (BMP)‐signaling and inflammation‐induced β‐cell failure in the context of diabetes. Results show that proinflammatory cytokines activate BMP signaling in human islets. Furthermore, enhanced BMP signaling reduces β‐cell marker expression, increases ER stress, and impairs β‐cell ...
Esmée Dekker, Javier Triñanes, Amadeo Muñoz Garcia, Natascha de Graaf, Eelco de Koning, Françoise Carlotti   +5 more
wiley   +1 more source

When Problems Become Solutions: Harnessing the Osteogenic Capacity of Disease-Causing Stem Cells to Repair Bone Fractures [PDF]

, 2022
While we often perceive disease as negative, there is potential to engineer seemingly negative biological phenomena into therapeutics to treat a variety of human illnesses.
Pasha, Mehreen
core   +2 more sources

Anaesthetic considerations in a child with fibrodysplasia ossificans progressiva [PDF]

, 2019
Fibrodysplasia ossificans progressiva (FOP) is an extremely rare genetic disorder characterised by extraskeletal ossification of connective tissue. Affected individuals often become completely immobilised by their third decade of life.
Kleyenstüber, Thomas
core   +1 more source

Spatiotemporal Analysis of Mesenchymal Stem Cells Fate Determination by Inflammatory Niche Following Soft Tissue Injury at a Single‐Cell Level

Advanced Science, Volume 11, Issue 43, November 20, 2024.
The dynamics of mesenchymal stem cell (MSC) lineage specification and associated dynamic regulation is highlighted by immune cells during HO formation, i.e., an elaborate trajectory of MSCs is characterized during aberrant osteochondrogenesis and demonstrates that MSCs proliferation are regulated by M1 macrophages (M1), neutrophils, and natural killer (
Chen Kan, Zhenya Tan, Haitao Wang, Wei Wang, Jiazhao Yang, Ya Zhang, Xiaoling Lu, Qirong Cheng, Lanyi Chai, Chao Peng, Jicheng Zhu, Chenghang Zhu, Hailin Wang, Li Zhan, Keqiong Lin, Yakun Liu, Lingqiang Zhang, Haitao Fan, Hong Zheng   +18 more
wiley   +1 more source

Fibrodysplasia ossificans progressiva-like in a cat

Arquivo Brasileiro de Medicina Veterinária e Zootecnia, 2012
Fibrodysplasia ossificans progressiva-like (FOP-like) was diagnosed in a young Brazilian cat presenting progressive lameness, pain upon manipulation and inability to extend the hind limbs.
L.Z. Crivelenti, S. Borin, A.M. Brum, D.K. Honsho   +3 more
doaj   +1 more source