Abnormal disulfide-linked oligomerization results in ER retention and altered signaling by TNFR1 mutants in TRAPS
Lobito AA, Kimberley FC, Muppidi JR, Komarow H, Jackson AJ, Hull KM, Kastner DL, Screaton GR, Siegel RM
Blood · 2006
Key Findings
- ●Mutant TNFR1 forms abnormal disulfide-linked oligomers retained in the ER
- ●Challenged the defective shedding hypothesis
- ●Proposed ER retention and misfolding as primary pathogenic mechanism
Referenced in (1 disease)
Validation history
Full log →No validation events recorded yet. Source will be checked on the next kipine-source-validator run.
ID: pmid-16684962DOI: 10.1182/blood-2005-11-006783PMID: 16684962