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Article

The Hajdu Cheney Mutation Is a Determinant of B-Cell Allocation of the Splenic Marginal Zone.

The American journal of pathology - 1 Jan 2018

Yu Jungeun, Zanotti Stefano, Walia Bhavita, Jellison Evan, Sanjay Archana, Canalis Ernesto

Abstract excerpt

The neurogenic locus notch homolog protein (Notch)-2 receptor is a determinant of B-cell allocation, and gain-of-NOTCH2-function mutations are associated with Hajdu-Cheney syndrome (HCS), a disease presenting with osteoporosis and acro-osteolysis. We generated a mouse model reproducing the HCS mutation (Notch2HCS), and heterozygous global mutant mice displayed gain-of-Notch2 function. In the mutant spleen, the...

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Share to B-Lymphocytes
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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

Open in PubMed Open full text

Identifiers

PMID: 29037852
DOI: 10.1016/j.ajpath.2017.09.010
PMCID: PMC5745525

Genes

NOTCH2

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026. Manifest publication-graph-cohort-2026-09-05.1.

Source receipt: pubmed, retrieved 5 Sept 2026.

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The Hajdu Cheney Mutation Is a Determinant of B-Cell Allocation of the Splenic Marginal Zone.PMID 29037852Read ↗

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Specific fibroblastic niches in secondary lymphoid organs orchestrate distinct Notch-regulated immune responses.Semantic proximity Read ↗

Sustained Notch2 signaling in osteoblasts, but not in osteoclasts, is linked to osteopenia in a mouse model of Hajdu-Cheney syndrome.Semantic proximity Read ↗

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