scholarly journals Characterization of the ventricular-subventricular stem cell niche during human brain development

Development ◽  
2018 ◽  
Vol 145 (20) ◽  
pp. dev170100 ◽  
Author(s):  
Amanda M. Coletti ◽  
Deepinder Singh ◽  
Saurabh Kumar ◽  
Tasnuva Nuhat Shafin ◽  
Patrick J. Briody ◽  
...  
Keyword(s):  
Stem Cell ◽  
Human Brain ◽  
Brain Development ◽  
Stem Cell Niche ◽  
Human Brain Development ◽  
Cell Niche

scholarly journals Characterization of drCol 15a1b: A Novel Component of the Stem Cell Niche in the Zebrafish Retina

Stem Cells ◽  
2010 ◽  
Vol 28 (8) ◽  
pp. 1399-1411 ◽  
Author(s):  
Veronica Gonzalez-Nunez ◽  
Valentina Nocco ◽  
Aidan Budd
Keyword(s):  
Stem Cell ◽  
Stem Cell Niche ◽  
Cell Niche

scholarly journals A human stem cell resource to decipher the biochemical and cellular basis of neurodevelopmental defects in Lowe Syndrome

Biology Open ◽  
2022 ◽  
Author(s):  
Bilal M. Akhtar ◽  
Priyanka Bhatia ◽  
Shubhra Acharya ◽  
Sanjeev Sharma ◽  
Yojet Sharma ◽  
...  
Keyword(s):  
Stem Cell ◽  
Lipid Metabolism ◽  
Human Brain ◽  
Brain Development ◽  
Cell Lines ◽  
Brain Function ◽  
Lowe Syndrome ◽  
Human Stem Cell ◽  
Human Brain Development ◽  
Developmental Features

Human brain development is a complex process where multiple cellular and developmental events are co-ordinated to generate normal structure and function. Alteration in any of these events can impact brain development, manifesting clinically as neurodevelopmental disorders. Human genetic disorders of lipid metabolism often present with features of altered brain function. Lowe syndrome (LS), is a X-linked recessive disease with features of altered brain function. LS results from mutations in OCRL1 that encodes a phosphoinositide 5-phosphatase enzyme. However, the cellular mechanisms by which loss of OCRL1 leads to brain defects remain unknown. Human brain development involves several cellular and developmental features not conserved in other species and understanding such mechanisms remains a challenge. Rodent models of LS have been generated, but failed to recapitulate features of the human disease. Here we describe the generation of human stem cell lines from LS patients. Further, we present biochemical characterization of lipid metabolism in patient cell lines and demonstrate their use as a “disease-in-a-dish” model for understanding the mechanism by which loss of OCRL1 leads to altered cellular and physiological brain development.

scholarly journals A human stem cell resource to decipher the biochemical and cellular basis of neurodevelopmental defects in Lowe Syndrome

2021 ◽  
Author(s):  
Bilal Akhtar ◽  
Priyanka Bhatia ◽  
Shubhra Acharya ◽  
Sanjeev Sharma ◽  
Yojet Sharma ◽  
...  
Keyword(s):  
Stem Cell ◽  
Lipid Metabolism ◽  
Human Brain ◽  
Brain Development ◽  
Cell Lines ◽  
Brain Function ◽  
Lowe Syndrome ◽  
Human Stem Cell ◽  
Human Brain Development ◽  
Developmental Features

Human brain development is a complex process where multiple cellular and developmental events are co-ordinated to generate normal structure and function. Alteration in any of these events can impact brain development, manifesting clinically as neurodevelopmental disorders. Human genetic disorders of lipid metabolism often present with features of altered brain function. Lowe syndrome (LS), is a X-linked recessive disease with features of altered brain function. LS results from mutations in OCRL1 that encodes a phosphoinositide 5-phosphatase enzyme. However, the cellular mechanisms by which loss of OCRL1 leads to brain defects remain unknown. Human brain development involves several cellular and developmental features not conserved in other species and understanding such mechanisms remains a challenge. Rodent models of LS have been generated, but failed to recapitulate features of the human disease. Here we describe the generation of human stem cell lines from LS patients. Further, we present biochemical characterization of lipid metabolism in patient cell lines and demonstrate their use as a disease-in-a-dish model for understanding the mechanism by which loss of OCRL1 leads to altered cellular and physiological brain development.

Characterization of the Limbal Epithelial Stem Cell Niche: Novel Imaging Techniques Permit In Vivo Observation and Targeted Biopsy of Limbal Epithelial Stem Cells

Stem Cells ◽  
2007 ◽  
Vol 25 (6) ◽  
pp. 1402-1409 ◽  
Author(s):  
Alex J. Shortt ◽  
Genevieve A. Secker ◽  
Peter M. Munro ◽  
Peng T. Khaw ◽  
Stephen J. Tuft ◽  
...  
Keyword(s):  
Stem Cells ◽  
Stem Cell ◽  
Stem Cell Niche ◽  
Imaging Techniques ◽  
Targeted Biopsy ◽  
Epithelial Stem Cells ◽  
Limbal Epithelial Stem Cells ◽  
Cell Niche

Characterization of the stem cell niche components within the seminiferous tubules in testicular biopsies of Klinefelter patients

2020 ◽  
Vol 113 (6) ◽  
pp. 1183-1195.e3 ◽  
Author(s):  
Dorien Van Saen ◽  
Veerle Vloeberghs ◽  
Inge Gies ◽  
Jean De Schepper ◽  
Herman Tournaye ◽  
...  
Keyword(s):  
Stem Cell ◽  
Stem Cell Niche ◽  
Seminiferous Tubules ◽  
Cell Niche ◽  
Niche Components
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