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Related Experiment Video

Updated: Aug 16, 2025

Isolation and Culture of Cells from the Nephrogenic Zone of the Embryonic Mouse Kidney

Isolation and Culture of Cells from the Nephrogenic Zone of the Embryonic Mouse Kidney

Published on: April 22, 2011

gldc Is Essential for Renal Progenitor Patterning during Kidney Development.

Nicole E Weaver¹, Allison Healy¹, Rebecca A Wingert¹

¹Department of Biological Sciences, Center for Stem Cells and Regenerative Medicine, Center for Zebrafish Research, Boler-Parseghian Center for Rare and Neglected Diseases, Warren Center for Drug Discovery, University of Notre Dame, Notre Dame, IN 46556, USA.

|December 23, 2022

Summary

Glycine decarboxylase (gldc) deficiency in zebrafish causes kidney and neural defects, mimicking nonketotic hyperglycinemia. This highlights gldc

Keywords:

NKH gldc glycine kidney nephron nonketotic hyperglycinemia

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In Situ Hybridization in Zebrafish Larvae and Juveniles during Mesonephros Development

In Situ Hybridization in Zebrafish Larvae and Juveniles during Mesonephros Development

Published on: August 13, 2021

Area of Science:

Developmental Biology
Biochemistry
Genetics

Background:

The glycine cleavage system (GCS) regulates glycine levels and one-carbon metabolism.
Mutations in glycine decarboxylase (gldc) cause nonketotic hyperglycinemia (NKH), a severe human disease.
Understanding gldc dysfunction's developmental impact is crucial.

Purpose of the Study:

To establish and characterize a zebrafish model for gldc deficiency.
To investigate the role of gldc in embryonic development, particularly renal and neural systems.
To elucidate the molecular mechanisms underlying gldc deficiency-related defects.

Main Methods:

Zebrafish gldc knockdown model creation.
Phenotypic analysis including morphology, fluid homeostasis, and neural development.
Whole mount in situ hybridization (WISH) for gene expression analysis.
Exogenous glycine treatment to assess glycine's direct effects.

Main Results:

Zebrafish gldc deficiency recapitulated NKH phenotypes, including renal and craniofacial abnormalities.
gldc transcripts were highly expressed in embryonic kidneys, with disrupted nephron development.
Renal progenitor populations were altered, indicating gldc's role in kidney patterning.
Exogenous glycine mimicked renal and cloacal defects, suggesting glycine level modulation is key.

Conclusions:

Zebrafish serve as a valuable model for studying gldc deficiency and NKH.
gldc is essential for proper renal progenitor development and kidney organogenesis.
Glycine level regulation by gldc is critical for preventing developmental abnormalities.