A hypomorphic model of CPS1 deficiency for investigating the effects of hyperammonemia on the developing nervous system

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Main Authors: Stuti Bakshi, Taryn Diep, Brandon J. Willis, Rachel Reyes, Grace F. Wu, Georgios Makris, Martin Poms, Isabel Day, Qin Sun, Irina Zhuravka, Lindsay Lueptow, Michelle Tang, Gareth A. Cromie, Aimée M. Dudley, Johannes Häberle, Gerald S. Lipshutz
Format: Article
Language:English
Published: The Company of Biologists 2025-07-01
Series:Disease Models & Mechanisms
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Online Access:http://dmm.biologists.org/content/18/7/dmm052303
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author Stuti Bakshi
Taryn Diep
Brandon J. Willis
Rachel Reyes
Grace F. Wu
Georgios Makris
Martin Poms
Isabel Day
Qin Sun
Irina Zhuravka
Lindsay Lueptow
Michelle Tang
Gareth A. Cromie
Aimée M. Dudley
Johannes Häberle
Gerald S. Lipshutz
author_facet Stuti Bakshi
Taryn Diep
Brandon J. Willis
Rachel Reyes
Grace F. Wu
Georgios Makris
Martin Poms
Isabel Day
Qin Sun
Irina Zhuravka
Lindsay Lueptow
Michelle Tang
Gareth A. Cromie
Aimée M. Dudley
Johannes Häberle
Gerald S. Lipshutz
author_sort Stuti Bakshi
collection DOAJ
format Article
id doaj-art-de39ddbfe4774f6bbe697aa35d6fc4d4
institution Kabale University
issn 1754-8403
1754-8411
language English
publishDate 2025-07-01
publisher The Company of Biologists
record_format Article
series Disease Models & Mechanisms
spelling doaj-art-de39ddbfe4774f6bbe697aa35d6fc4d42025-08-20T03:41:21ZengThe Company of BiologistsDisease Models & Mechanisms1754-84031754-84112025-07-0118710.1242/dmm.052303052303A hypomorphic model of CPS1 deficiency for investigating the effects of hyperammonemia on the developing nervous systemStuti Bakshi0Taryn Diep1Brandon J. Willis2Rachel Reyes3Grace F. Wu4Georgios Makris5Martin Poms6Isabel Day7Qin Sun8Irina Zhuravka9Lindsay Lueptow10Michelle Tang11Gareth A. Cromie12Aimée M. Dudley13Johannes Häberle14Gerald S. Lipshutz15 Departments of Surgery, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA Departments of Surgery, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA Mouse Biology Program, University of California, Davis, Davis, CA 95618, USA Departments of Surgery, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA Departments of Surgery, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA Division of Metabolism and Children's Research Center, University Children's Hospital Zurich, Zurich 8008, Switzerland Clinical Chemistry and Biochemistry, University Children's Hospital Zurich, Zurich 8008, Switzerland Departments of Surgery, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA Department of Psychology, UCLA, Los Angeles, CA 90095, USA Department of Psychology, UCLA, Los Angeles, CA 90095, USA Pacific Northwest Research Institute, Seattle, WA 98122, USA Pacific Northwest Research Institute, Seattle, WA 98122, USA Pacific Northwest Research Institute, Seattle, WA 98122, USA Division of Metabolism and Children's Research Center, University Children's Hospital Zurich, Zurich 8008, Switzerland Departments of Surgery, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA http://dmm.biologists.org/content/18/7/dmm052303carbamoyl phosphate synthetase 1 deficiencyhuman mutationenzyme activityhyperammonemiaglutamine
spellingShingle Stuti Bakshi
Taryn Diep
Brandon J. Willis
Rachel Reyes
Grace F. Wu
Georgios Makris
Martin Poms
Isabel Day
Qin Sun
Irina Zhuravka
Lindsay Lueptow
Michelle Tang
Gareth A. Cromie
Aimée M. Dudley
Johannes Häberle
Gerald S. Lipshutz
A hypomorphic model of CPS1 deficiency for investigating the effects of hyperammonemia on the developing nervous system
Disease Models & Mechanisms
carbamoyl phosphate synthetase 1 deficiency
human mutation
enzyme activity
hyperammonemia
glutamine
title A hypomorphic model of CPS1 deficiency for investigating the effects of hyperammonemia on the developing nervous system
title_full A hypomorphic model of CPS1 deficiency for investigating the effects of hyperammonemia on the developing nervous system
title_fullStr A hypomorphic model of CPS1 deficiency for investigating the effects of hyperammonemia on the developing nervous system
title_full_unstemmed A hypomorphic model of CPS1 deficiency for investigating the effects of hyperammonemia on the developing nervous system
title_short A hypomorphic model of CPS1 deficiency for investigating the effects of hyperammonemia on the developing nervous system
title_sort hypomorphic model of cps1 deficiency for investigating the effects of hyperammonemia on the developing nervous system
topic carbamoyl phosphate synthetase 1 deficiency
human mutation
enzyme activity
hyperammonemia
glutamine
url http://dmm.biologists.org/content/18/7/dmm052303
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