Characterization of Notch1 antibodies that inhibit signaling of both normal and mutated Notch1 receptors.

<h4>Background</h4>Notch receptors normally play a key role in guiding a variety of cell fate decisions during development and differentiation of metazoan organisms. On the other hand, dysregulation of Notch1 signaling is associated with many different types of cancer as well as tumor an...

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Main Authors: Miguel Aste-Amézaga, Ningyan Zhang, Janet E Lineberger, Beth A Arnold, Timothy J Toner, Mingcheng Gu, Lingyi Huang, Salvatore Vitelli, Kim T Vo, Peter Haytko, Jing Zhang Zhao, Frederic Baleydier, Sarah L'Heureux, Hongfang Wang, Wendy R Gordon, Elizabeth Thoryk, Marie Blanke Andrawes, Kittichoat Tiyanont, Kimberly Stegmaier, Giovanni Roti, Kenneth N Ross, Laura L Franlin, Hui Wang, Fubao Wang, Michael Chastain, Andrew J Bett, Laurent P Audoly, Jon C Aster, Stephen C Blacklow, Hans E Huber
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2010-02-01
Series:PLoS ONE
Online Access:https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0009094&type=printable
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author Miguel Aste-Amézaga
Ningyan Zhang
Janet E Lineberger
Beth A Arnold
Timothy J Toner
Mingcheng Gu
Lingyi Huang
Salvatore Vitelli
Kim T Vo
Peter Haytko
Jing Zhang Zhao
Frederic Baleydier
Sarah L'Heureux
Hongfang Wang
Wendy R Gordon
Elizabeth Thoryk
Marie Blanke Andrawes
Kittichoat Tiyanont
Kimberly Stegmaier
Giovanni Roti
Kenneth N Ross
Laura L Franlin
Hui Wang
Fubao Wang
Michael Chastain
Andrew J Bett
Laurent P Audoly
Jon C Aster
Stephen C Blacklow
Hans E Huber
author_facet Miguel Aste-Amézaga
Ningyan Zhang
Janet E Lineberger
Beth A Arnold
Timothy J Toner
Mingcheng Gu
Lingyi Huang
Salvatore Vitelli
Kim T Vo
Peter Haytko
Jing Zhang Zhao
Frederic Baleydier
Sarah L'Heureux
Hongfang Wang
Wendy R Gordon
Elizabeth Thoryk
Marie Blanke Andrawes
Kittichoat Tiyanont
Kimberly Stegmaier
Giovanni Roti
Kenneth N Ross
Laura L Franlin
Hui Wang
Fubao Wang
Michael Chastain
Andrew J Bett
Laurent P Audoly
Jon C Aster
Stephen C Blacklow
Hans E Huber
author_sort Miguel Aste-Amézaga
collection DOAJ
description <h4>Background</h4>Notch receptors normally play a key role in guiding a variety of cell fate decisions during development and differentiation of metazoan organisms. On the other hand, dysregulation of Notch1 signaling is associated with many different types of cancer as well as tumor angiogenesis, making Notch1 a potential therapeutic target.<h4>Principal findings</h4>Here we report the in vitro activities of inhibitory Notch1 monoclonal antibodies derived from cell-based and solid-phase screening of a phage display library. Two classes of antibodies were found, one directed against the EGF-repeat region that encompasses the ligand-binding domain (LBD), and the second directed against the activation switch of the receptor, the Notch negative regulatory region (NRR). The antibodies are selective for Notch1, inhibiting Jag2-dependent signaling by Notch1 but not by Notch 2 and 3 in reporter gene assays, with EC(50) values as low as 5+/-3 nM and 0.13+/-0.09 nM for the LBD and NRR antibodies, respectively, and fail to recognize Notch4. While more potent, NRR antibodies are incomplete antagonists of Notch1 signaling. The antagonistic activity of LBD, but not NRR, antibodies is strongly dependent on the activating ligand. Both LBD and NRR antibodies bind to Notch1 on human tumor cell lines and inhibit the expression of sentinel Notch target genes, including HES1, HES5, and DTX1. NRR antibodies also strongly inhibit ligand-independent signaling in heterologous cells transiently expressing Notch1 receptors with diverse NRR "class I" point mutations, the most common type of mutation found in human T-cell acute lymphoblastic leukemia (T-ALL). In contrast, NRR antibodies failed to antagonize Notch1 receptors bearing rare "class II" or "class III" mutations, in which amino acid insertions generate a duplicated or constitutively sensitive metalloprotease cleavage site. Signaling in T-ALL cell lines bearing class I mutations is partially refractory to inhibitory antibodies as compared to cell-penetrating gamma-secretase inhibitors.<h4>Conclusions/significance</h4>Antibodies that compete with Notch1 ligand binding or that bind to the negative regulatory region can act as potent inhibitors of Notch1 signaling. These antibodies may have clinical utility for conditions in which inhibition of signaling by wild-type Notch1 is desired, but are likely to be of limited value for treatment of T-ALLs associated with aberrant Notch1 activation.
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spelling doaj-art-b4ac98e727c04ff3ad08212b68b47d082025-08-20T02:31:50ZengPublic Library of Science (PLoS)PLoS ONE1932-62032010-02-0152e909410.1371/journal.pone.0009094Characterization of Notch1 antibodies that inhibit signaling of both normal and mutated Notch1 receptors.Miguel Aste-AmézagaNingyan ZhangJanet E LinebergerBeth A ArnoldTimothy J TonerMingcheng GuLingyi HuangSalvatore VitelliKim T VoPeter HaytkoJing Zhang ZhaoFrederic BaleydierSarah L'HeureuxHongfang WangWendy R GordonElizabeth ThorykMarie Blanke AndrawesKittichoat TiyanontKimberly StegmaierGiovanni RotiKenneth N RossLaura L FranlinHui WangFubao WangMichael ChastainAndrew J BettLaurent P AudolyJon C AsterStephen C BlacklowHans E Huber<h4>Background</h4>Notch receptors normally play a key role in guiding a variety of cell fate decisions during development and differentiation of metazoan organisms. On the other hand, dysregulation of Notch1 signaling is associated with many different types of cancer as well as tumor angiogenesis, making Notch1 a potential therapeutic target.<h4>Principal findings</h4>Here we report the in vitro activities of inhibitory Notch1 monoclonal antibodies derived from cell-based and solid-phase screening of a phage display library. Two classes of antibodies were found, one directed against the EGF-repeat region that encompasses the ligand-binding domain (LBD), and the second directed against the activation switch of the receptor, the Notch negative regulatory region (NRR). The antibodies are selective for Notch1, inhibiting Jag2-dependent signaling by Notch1 but not by Notch 2 and 3 in reporter gene assays, with EC(50) values as low as 5+/-3 nM and 0.13+/-0.09 nM for the LBD and NRR antibodies, respectively, and fail to recognize Notch4. While more potent, NRR antibodies are incomplete antagonists of Notch1 signaling. The antagonistic activity of LBD, but not NRR, antibodies is strongly dependent on the activating ligand. Both LBD and NRR antibodies bind to Notch1 on human tumor cell lines and inhibit the expression of sentinel Notch target genes, including HES1, HES5, and DTX1. NRR antibodies also strongly inhibit ligand-independent signaling in heterologous cells transiently expressing Notch1 receptors with diverse NRR "class I" point mutations, the most common type of mutation found in human T-cell acute lymphoblastic leukemia (T-ALL). In contrast, NRR antibodies failed to antagonize Notch1 receptors bearing rare "class II" or "class III" mutations, in which amino acid insertions generate a duplicated or constitutively sensitive metalloprotease cleavage site. Signaling in T-ALL cell lines bearing class I mutations is partially refractory to inhibitory antibodies as compared to cell-penetrating gamma-secretase inhibitors.<h4>Conclusions/significance</h4>Antibodies that compete with Notch1 ligand binding or that bind to the negative regulatory region can act as potent inhibitors of Notch1 signaling. These antibodies may have clinical utility for conditions in which inhibition of signaling by wild-type Notch1 is desired, but are likely to be of limited value for treatment of T-ALLs associated with aberrant Notch1 activation.https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0009094&type=printable
spellingShingle Miguel Aste-Amézaga
Ningyan Zhang
Janet E Lineberger
Beth A Arnold
Timothy J Toner
Mingcheng Gu
Lingyi Huang
Salvatore Vitelli
Kim T Vo
Peter Haytko
Jing Zhang Zhao
Frederic Baleydier
Sarah L'Heureux
Hongfang Wang
Wendy R Gordon
Elizabeth Thoryk
Marie Blanke Andrawes
Kittichoat Tiyanont
Kimberly Stegmaier
Giovanni Roti
Kenneth N Ross
Laura L Franlin
Hui Wang
Fubao Wang
Michael Chastain
Andrew J Bett
Laurent P Audoly
Jon C Aster
Stephen C Blacklow
Hans E Huber
Characterization of Notch1 antibodies that inhibit signaling of both normal and mutated Notch1 receptors.
PLoS ONE
title Characterization of Notch1 antibodies that inhibit signaling of both normal and mutated Notch1 receptors.
title_full Characterization of Notch1 antibodies that inhibit signaling of both normal and mutated Notch1 receptors.
title_fullStr Characterization of Notch1 antibodies that inhibit signaling of both normal and mutated Notch1 receptors.
title_full_unstemmed Characterization of Notch1 antibodies that inhibit signaling of both normal and mutated Notch1 receptors.
title_short Characterization of Notch1 antibodies that inhibit signaling of both normal and mutated Notch1 receptors.
title_sort characterization of notch1 antibodies that inhibit signaling of both normal and mutated notch1 receptors
url https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0009094&type=printable
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