Nuclear transcription factor kB (NF-kB) activity in lymphocyte populations in children with Wilson-Konovalov disease

نویسندگان

چکیده

Wilson's disease (WD) is a rare hereditary caused by deficiency of the ATF7B transporter. The accumulation copper can cause damage to organs and cells, mainly liver. Copper exposure modulate cytokine synthesis through molecular cellular signaling pathways, including nuclear transcription factor NF-kB pathway. main regulator inflammation cell death, acts as central link between liver damage, fibrosis hepatocellular carcinoma. An excess NF-kB-dependent response stimulates inflammatory reactions, but excessive inhibition negatively affect viability hepatocytes. Method flow cytometry with visualization — Amnis ImageStream X allows evaluate activity (% activated cells in populations). aim: lymphocyte populations children WD disease. Immunophenotyping lymphocytes assessment level translocation were performed 52 25 comparison group. mass concentration daily urine was determined atomic absorption method using AAnalyst 800 spectrometer. In WD, content varied from 5 90% depending on population; highest detected B 57.5 (37-68) %. A significant difference distributions number healthy shown (F-criterion, p < 0.01). most cases, are characterized decrease (in 43% cases), T helper (48%), cytotoxic (44%) Th17 (41%). 9.7 2582 mcg/day, Me = 616 (210-1173). direct relationship obtained lymphocytes, r 0.34, 0.016. correlates biochemical markers severity (ALT, AST, GGT) urine. study pathway seems promising for better understanding pathogenetic mechanisms formation WD.

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ژورنال

عنوان ژورنال: ??????????? ???????????

سال: 2023

ISSN: ['2409-5788']

DOI: https://doi.org/10.15789/1563-0625-ntf-2799