Mutant calreticulin in myeloproliferative neoplasms.
Journal
Blood
ISSN: 1528-0020
Titre abrégé: Blood
Pays: United States
ID NLM: 7603509
Informations de publication
Date de publication:
19 12 2019
19 12 2019
Historique:
received:
12
08
2019
accepted:
19
09
2019
pubmed:
29
9
2019
medline:
25
3
2020
entrez:
29
9
2019
Statut:
ppublish
Résumé
Recurrent mutations in calreticulin are present in ∼20% of patients with myeloproliferative neoplasms (MPNs). Since its discovery in 2013, we now have a more precise understanding of how mutant CALR, an endoplasmic reticulum chaperone protein, activates the JAK/STAT signaling pathway via a pathogenic binding interaction with the thrombopoietin receptor MPL to induce MPNs. In this Spotlight article, we review the current understanding of the biology underpinning mutant CALR-driven MPNs, discuss clinical implications, and highlight future therapeutic approaches.
Identifiants
pubmed: 31562135
pii: S0006-4971(20)73128-9
doi: 10.1182/blood.2019000622
pmc: PMC6923668
doi:
Substances chimiques
CALR protein, human
0
Calreticulin
0
Neoplasm Proteins
0
Receptors, Thrombopoietin
0
STAT Transcription Factors
0
MPL protein, human
143641-95-6
Janus Kinases
EC 2.7.10.2
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
2242-2248Subventions
Organisme : NCI NIH HHS
ID : K12 CA087723
Pays : United States
Organisme : NCI NIH HHS
ID : P50 CA206963
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL131835
Pays : United States
Informations de copyright
© 2019 by The American Society of Hematology.
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