Inositol-requiring enzyme 1α/X-box protein 1 pathway expression is impaired in pediatric cholestatic liver disease explants.

Comparing gene expression of liver IRE1α , XBP1s , EDEM1 and ERdj4 of the two pCLD subgroups (PFIC, ALGS) showed a trend toward lower gene expression in the PFIC compared to ALGS samples, however, none of these differences were statistically significant and all were lower than the pNormal controls ( Fig 2B ).

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In addition, 4 other highly significant pathways included ‘NABA CORE MATRISOME’, the reactome pathway: ‘Extracellular matrix organization’, and the GO Biological Processes pathways: regulation of cell adhesion’ and ‘chemotaxis’.

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