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Essential cell-extrinsic requirement for PDIA6 in lymphoid and myeloid development.

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Date
2020-04
Authors
Choi, Jin Huk
Zhong, Xue
Zhang, Zhao
Su, Lijing
McAlpine, William
Misawa, Takuma
Liao, Tzu-Chieh
Zhan, Xiaoming
Russell, Jamie
Ludwig, Sara
Li, Xiaohong
Tang, Miao
Anderton, Priscilla
Moresco, Eva Marie Y
Beutler, Bruce
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(15 total)
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Abstract
In a forward genetic screen of N-ethyl-N-nitrosourea (ENU)-induced mutant mice for aberrant immune function, we identified mice with a syndromic disorder marked by growth retardation, diabetes, premature death, and severe lymphoid and myeloid hypoplasia together with diminished T cell-independent (TI) antibody responses. The causative mutation was in Pdia6, an essential gene encoding protein disulfide isomerase A6 (PDIA6), an oxidoreductase that functions in nascent protein folding in the endoplasmic reticulum. The immune deficiency caused by the Pdia6 mutation was, with the exception of a residual T cell developmental defect, completely rescued in irradiated wild-type recipients of PDIA6-deficient bone marrow cells, both in the absence or presence of competition. The viable hypomorphic allele uncovered in these studies reveals an essential role for PDIA6 in hematopoiesis, but one extrinsic to cells of the hematopoietic lineage. We show evidence that this role is in the proper folding of Wnt3a, BAFF, IL-7, and perhaps other factors produced by the extra-hematopoietic compartment that contribute to the development and lineage commitment of hematopoietic cells.
Type
Journal article
Subject
Lymphocytes
T-Lymphocytes
Cell Line
Myeloid Cells
Animals
Mice, Inbred C57BL
Humans
Mice
Interleukin-7
Hematopoiesis
Female
Male
B-Cell Activating Factor
Protein Disulfide-Isomerases
HEK293 Cells
Wnt3A Protein
Permalink
https://hdl.handle.net/10161/23464
Published Version (Please cite this version)
10.1084/jem.20190006
Publication Info
Choi, Jin Huk; Zhong, Xue; Zhang, Zhao; Su, Lijing; McAlpine, William; Misawa, Takuma; ... Beutler, Bruce (2020). Essential cell-extrinsic requirement for PDIA6 in lymphoid and myeloid development. The Journal of experimental medicine, 217(4). 10.1084/jem.20190006. Retrieved from https://hdl.handle.net/10161/23464.
This is constructed from limited available data and may be imprecise. To cite this article, please review & use the official citation provided by the journal.
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Scholars@Duke

Liao

Tzu-chieh Liao

Student
Program Start Year: 2021Rotating Student
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