Sonic Hedgehog-Gli1 Signaling and Cellular Retinoic Acid Binding Protein 1 Gene Regulation in Motor Neuron Differentiation and Diseases.

International Journal of Molecular Sciences
Yu-Lung LinLi-Na Wei

Abstract

Cellular retinoic acid-binding protein 1 (CRABP1) is highly expressed in motor neurons. Degenerated motor neuron-like MN1 cells are engineered by introducing SODG93A or AR-65Q to model degenerated amyotrophic lateral sclerosis (ALS) or spinal bulbar muscular atrophy neurons. Retinoic acid (RA)/sonic hedgehog (Shh)-induced embryonic stem cells differentiation into motor neurons are employed to study up-regulation of Crabp1 by Shh. In SODG93A or AR-65Q MN1 neurons, CRABP1 level is reduced, revealing a correlation of motor neuron degeneration with Crabp1 down-regulation. Up-regulation of Crabp1 by Shh is mediated by glioma-associated oncogene homolog 1 (Gli1) that binds the Gli target sequence in Crabp1's neuron-specific regulatory region upstream of minimal promoter. Gli1 binding triggers chromatin juxtaposition with minimal promoter, activating transcription. Motor neuron differentiation and Crabp1 up-regulation are both inhibited by blunting Shh with Gli inhibitor GANT61. Expression data mining of ALS and spinal muscular atrophy (SMA) motor neurons shows reduced CRABP1, coincided with reduction in Shh-Gli1 signaling components. This study reports motor neuron degeneration correlated with down-regulation in Crabp1 and Shh-Gli si...Continue Reading

References

Nov 1, 1989·Proceedings of the National Academy of Sciences of the United States of America·A V Perez-CastroM C Nguyen-Huu
Dec 1, 1994·Differentiation; Research in Biological Diversity·D R de BruijnA Geurts van Kessel
Sep 13, 1994·Proceedings of the National Academy of Sciences of the United States of America·P GorryP Chambon
Oct 3, 1999·Progress in Nucleic Acid Research and Molecular Biology·J L Napoli
Aug 15, 2002·Cell·Hynek WichterleThomas M Jessell
Dec 7, 2002·Nature Neuroscience·Karen LaiDavid V Schaffer
Mar 17, 2004·Brain Research. Gene Expression Patterns·F C Zhou, L N Wei
May 12, 2006·Journal of Neurobiology·Malcolm Maden
Sep 13, 2007·Science's STKE : Signal Transduction Knowledge Environment·Jong-Sun KangRobert S Krauss
Dec 12, 2012·Nature Biotechnology·Cole TrapnellLior Pachter
Jun 14, 2014·Neurobiology of Disease·Christopher GrunseichKenneth H Fischbeck
Jan 7, 2015·Cellular and Molecular Life Sciences : CMLS·Amanda JanesickBruce Blumberg
Dec 15, 2015·Gene·Simran J KaurShazia Rashid
Nov 21, 2016·Trends in Neurosciences·Pamela J YaoMark P Mattson
Dec 10, 2016·Journal of Developmental Biology·Yesser H BelgacemLaura N Borodinsky
Aug 18, 2018·International Journal of Molecular Sciences·Shang-Der ChenDing-I Yang
Jun 6, 2019·International Journal of Obesity : Journal of the International Association for the Study of Obesity·Yi-Wei LinLi-Na Wei
Jul 31, 2019·Scientific Reports·Sung Wook ParkLi-Na Wei

❮ Previous
Next ❯

Citations


❮ Previous
Next ❯

Datasets Mentioned

BETA
GSE108094

Methods Mentioned

BETA
SMA
immunoprecipitation
ChIP
PCR
RNA-seq
transfection
SMO

Software Mentioned

CuffDiff2
GenePix Pro

Related Concepts

Related Feeds

Amyloid Lateral Sclerosis

Amyotrophic Lateral Sclerosis (ALS) is a progressive nervous system disease associated with the death of neurons that control voluntary muscles. Discover the latest research on ALS here.