Article
Type I but Not Type II Calreticulin Mutations Activate the IRE1α/XBP1 Pathway of the Unfolded Protein Response to Drive Myeloproliferative Neoplasms.
Blood cancer discovery - 6 Jul 2022
Ibarra Juan, Elbanna Yassmin A, Kurylowicz Katarzyna, Ciboddo Michele, Greenbaum Harrison S, Arellano Nicole S, Rodriguez Deborah, Evers Maria, Bock-Hughes Althea, Liu Chenyu, Smith Quinn, Lutze Julian, Baumeister Julian, Kalmer Milena, Olschok Kathrin, Nicholson Benjamin, Silva Diane, Maxwell Luke, Dowgielewicz Jonathan, Rumi Elisa, Pietra Daniela, Casetti Ilaria Carola, Catricala Silvia, Koschmieder Steffen, Gurbuxani Sandeep, Schneider Rebekka K, Oakes Scott A, Elf Shannon E
Abstract excerpt
Approximately 20% of patients with myeloproliferative neoplasms (MPN) harbor mutations in the gene calreticulin (CALR), with 80% of those mutations classified as either type I or type II. While type II CALR-mutant proteins retain many of the Ca2+ binding sites present in the wild-type protein, type I CALR-mutant proteins lose these residues. The functional consequences of this differential loss of Ca2+ binding...
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