Current and Future Therapeutical Aspects of X-Linked Hypophosphatemia in Children With Special Attention to Klotho/Fibroblast Growth Factor 23 System

Bittmann, Stefan; Moschüring-Alieva, Elena; Luchter, Elisabeth; Villalon, Gloria

doi:10.14740/jocmr4820

Cited by 1 publication

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References 31 publications

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“…Erk1/2 and STAT 1 seem to regulate FGFR3 mediated inhibition of chondrocyte proliferation [51,52]. FGFR1 is part of complex binding with FGF23 and Klotho in Xlinked hypophosphatemia [53]. Klotho-FGFR1-FGF23 trimeric complex signaling plays an important role in X-linked hypophosphatemia in childhood and inhibition of this complex could diminish the level of FGF in the kidney in this disease [53].…”

Section: Intracellular Signaling With Protein Interactions (Modulator...mentioning

confidence: 99%

The Role of Fibroblast Growth Factor-Receptor Pathway Aberrations in Pediatric Diseases

Bittmann,

Luchter,

Moschüring-Alieva

2024

AJPR

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The fibroblast growth factor receptors play a crucial role in binding to fibroblast growth factor and are involved in various pathological conditions. These receptors consist of an extracellular ligand domain, a transmembrane helix domain, and an intracellular domain with tyrosine kinase activity. There are over 48 different isoforms of FGFR. Each FGF receptor in ligand-binding properties and kinase domains. The FGF/FGFR signaling pathway is implicated in various pediatric cancers and therefore are both non-selective and selective FGFR inhibitors available. Additionally, there are five distinct membrane FGF receptors dentified in vertebrates. All belonging to the tyrosine kinase superfamily. In this manuscript, the focus is based on an analysis of the role of FGF receptor signaling pathways and aberrations especially in pediatric diseases. Erk 1 and Erk 2 seem to have an important role as a mediator of FGF signaling in different biological and developmental processes. FGF signaling plays an important role in conserved developmental functions in skeletal growth, palate closure, ear development, cranial suture ossification and neuronal development in the child. Aberrant FGF signaling causes different congenital disorders and different forms of cancer in childhood. Modulating FGF signaling is of great importance for the treatment of rare diseases in childhood.

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Section: Intracellular Signaling With Protein Interactions (Modulator...mentioning

confidence: 99%

The Role of Fibroblast Growth Factor-Receptor Pathway Aberrations in Pediatric Diseases

Bittmann,

Luchter,

Moschüring-Alieva

2024

AJPR

View full text Add to dashboard Cite

show abstract

Current and Future Therapeutical Aspects of X-Linked Hypophosphatemia in Children With Special Attention to Klotho/Fibroblast Growth Factor 23 System

Cited by 1 publication

References 31 publications

The Role of Fibroblast Growth Factor-Receptor Pathway Aberrations in Pediatric Diseases

The Role of Fibroblast Growth Factor-Receptor Pathway Aberrations in Pediatric Diseases

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