Carbimazole

McCune-Albright syndrome (MAS) is somatic mosaicism disorder due to mutations in GNAS gene leading to a combination of fibrous dysplasia of bone, café-au-lait skin lesion and hyperfunctioning endocrinopathies. We report an 11-year-old boy who presented with bone pains and easy fractures with minor traumas that were treated with surgeries. He had multiple café-au-lait lesions, hypertrophic scars of prior surgeries and deformities of bones on examination and primary hyperthyroidism on investigation. His condition was initially misdiagnosed as osteogenesis imperfecta but the finding of hyperthyroidism reaffirms the diagnosis of MAS. He was treated with pamidronate, carbimazole and surgical operations to correct bony deformities.

In this study, the anti-aging potential of melibiose was examined, and its molecular mechanism was elucidated using Caenorhabditis elegans as a model organism. The findings demonstrated that melibiose at concentrations of 100 μM, 150 μM, and 200 μM significantly increased nematode lifespan by 15.38%, 23.08%, and 30.77% respectively. Additionally, melibiose enhanced resistance against heat and oxidative stress, improved nematode motility, reduced lipofuscin and reactive oxygen species (ROS) accumulation, and increased antioxidant enzyme activity. Through the use of gene-deletion nematodes, transgenic nematodes, RT-qPCR, and metabolomics, it was determined that melibiose potentially exerts its effects through multiple pathways including the insulin signaling pathway (down-regulation of daf-2 and age-1, up-regulation of sod-3 and hsp-16.2), the AMP-activated protein kinase (AMPK) pathway (up-regulation of aak-2), and the JNK pathway (up-regulation of jnk-1). Activation of transcription factors DAF-16, SKN-1, and HSF-1 was observed, moreover, delaying the aging process by promoting autophagy (upregulation of lgg-1 and bec-1) and mitochondrial function (upregulation of hsp-6, hsp-60, and mev-1) to resist oxidative damage. And its anti-aging signature metabolites may be Carbimazole, 4-Hydroxy-2-oxoglutaric acid, and 1,4-Dithiothreitol.