AI Article Synopsis
- Inherited metabolic liver diseases are genetic disorders that disrupt liver metabolism, mostly affecting children and impacting various metabolic pathways like iron and amino acids.
- Traditional research has focused on changes in liver cells, but new findings suggest these diseases also significantly impact the immune system.
- This review aims to highlight the immune-related aspects of these diseases and propose new treatment strategies, broadening the understanding of their mechanisms.
Article Abstract
Inherited metabolic liver diseases arise from genetic mutations that lead to disruptions in liver metabolic pathways and are predominantly observed in pediatric populations. The spectrum of genetic metabolic liver disorders is diverse, encompassing a range of conditions associated with aberrations in iron, copper, carbohydrate, lipid, protein, and amino acid metabolism. Historically, research in the domain of genetic metabolic liver diseases has predominantly concentrated on hepatic parenchymal cell alterations. Nevertheless, emerging studies suggest that inherited metabolic liver diseases exert significant influences on the immune microenvironment, both within the liver and systemically. This review endeavors to encapsulate the immunological features of genetic metabolic liver diseases, aiming to expand the horizons of researchers in this discipline, and to elucidate the underlying pathophysiological mechanisms pertinent to hereditary metabolic liver diseases and to propose innovative therapeutic approaches.
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Source |
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11438594 | PMC |
| http://dx.doi.org/10.4254/wjh.v16.i9.1258 | DOI Listing |
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