Why these kids can't make gut slime

Original Title

Human AGR2 Deficiency Causes Mucus Barrier Dysfunction and Infantile Inflammatory Bowel Disease

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Summary

Two brothers had terrible gut problems because their bodies couldn't make a slimy barrier that protects the intestines from bacteria.

Proposed Mechanism
AGR2 deficiency impairs MUC2 processing and triggers goblet cell ER stress
Verified
AGR2 deficiency compromises ER stress resolution independent of mucin processing
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Quality Analysis
Methodology
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Detailed descriptions of individual patients or small groups. Valuable for identifying new conditions or side effects, but cannot establish generalizable conclusions.

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Publication

Authors

Al-Shaibi AA, Abdel-Motal UM, Hubrack SZ, Bullock AN, Al-Marri AA, Agrebi N, Al-Subaiey AA, Ibrahim NA, Charles AK, COLORS in IBD-Qatar Study Group, Elawad M, Uhlig HH, Lo B