ArticleThe Journal of comparative neurology2013
Mutation of the BiP/GRP78 gene causes axon outgrowth and fasciculation defects in the thalamocortical connections of the mammalian forebrain.
Article in The Journal of comparative neurology, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 25 citations in OpenAlex.
- Hexosamine Pathway Disruption by GFPT1 Loss Drives Coordinated Defects in Glycosylation, Autophagy, and Trafficking.Biomolecules · 2026Article
- Impact of ATF6 deletion on the embryonic brain development.iScience · 2025Article
- The expression of transcription factors in the human fetal subthalamic nucleus suggests its origin from the first hypothalamic prosomere.Brain structure & function · 2025Article
- Phenotypic Characterization and Brain Structure Analysis of Calcium Channel Subunit αFrontiers in synaptic neuroscience · 2021Article
- Cerebral Dopamine Neurotrophic Factor Regulates Multiple Neuronal Subtypes and Behavior.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2020Article
- Endoplasmic reticulum and Golgi stress in microcephaly.Cell stress · 2019Review
- Molecular guidance cues in the development of visual pathway.Protein & cell · 2018Review
- ALS-Associated Endoplasmic Reticulum Proteins in Denervated Skeletal Muscle: Implications for Motor Neuron Disease Pathology.Brain pathology (Zurich, Switzerland) · 2017Article
- Neural Stem Cells to Cerebral Cortex: Emerging Mechanisms Regulating Progenitor Behavior and Productivity.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2016Review
- Endoplasmic Reticulum Protein Quality Control Failure in Myelin Disorders.Frontiers in molecular neuroscience · 2016Review
- The Unfolded Protein Response and Cholesterol Biosynthesis Link Luman/CREB3 to Regenerative Axon Growth in Sensory Neurons.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2015Article
- Role of the unfolded protein response, GRP78 and GRP94 in organ homeostasis.Journal of cellular physiology · 2015Review
- Oxidative stress, unfolded protein response, and apoptosis in developmental toxicity.International review of cell and molecular biology · 2015Review
- Loop formation and self-fasciculation of cortical axon using photonic guidance at long working distance.Scientific reports · 2014Article
- Semi-automatic quantification of neurite fasciculation in high-density neurite images by the neurite directional distribution analysis (NDDA).Journal of neuroscience methods · 2014Article
- Protein quality control in the endoplasmic reticulum.F1000prime reports · 2014Review
- The vertebrate-specific Kinesin-6, Kif20b, is required for normal cytokinesis of polarized cortical stem cells and cerebral cortex size.Development (Cambridge, England) · 2013Article
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
Abstract
Proper development of axonal connections is essential for brain function. A forward genetic screen for mice with defects in thalamocortical development previously isolated a mutant called baffled. Here we describe the axonal defects of baffled in further detail and identify a point mutation in the Hspa5 gene, encoding the endoplasmic reticulum chaperone BiP/GRP78. This hypomorphic mutation of BiP disrupts proper development of the thalamocortical axon projection and other forebrain axon tracts, as well as cortical lamination. In baffled mutant brains, a reduced number of thalamic axons innervate the cortex by the time of birth. Thalamocortical and corticothalamic axons are delayed, overfasciculated, and disorganized along their pathway through the ventral telencephalon. Furthermore, dissociated mutant neurons show reduced axon extension in vitro. Together, these findings demonstrate a sensitive requirement for the endoplasmic reticulum chaperone BiP/GRP78 during axon outgrowth and pathfinding in the developing mammalian brain.
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