ReviewProgress in brain research2012
A Sonic hedgehog (Shh) response deficit in trisomic cells may be a common denominator for multiple features of Down syndrome.
Review in Progress in brain research, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers, 1 of them a synthesis that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
28 citing papers in PubMed, 1 synthesis or guideline pooled it, 36 citations in OpenAlex.
- Craniofacial syndromes and class III phenotype: common genotype fingerprints? A scoping review and meta-analysis.Pediatric research · 2024Pooled it
- Aberrant PCM1 accumulation in Trisomy 21 mislocalizes E3 ligases, delaying primary ciliogenesis.bioRxiv : the preprint server for biology · 2026Article
- A Researcher's guide to rodent models of Down syndrome: Recent insights and translational perspectives.STAR protocols · 2026Review
- Integrated analysis of immunometabolic interactions in Down syndrome.Science advances · 2024Article
- Overexpression screen of chromosome 21 genes reveals modulators of Sonic hedgehog signaling relevant to Down syndrome.Disease models & mechanisms · 2023Article
- Shaking up the silence: consequences of HMGN1 antagonizing PRC2 in the Down syndrome brain.Epigenetics & chromatin · 2022Review
- Trisomy 21 increases microtubules and disrupts centriolar satellite localization.Molecular biology of the cell · 2022Article
- Forebrain Shh overexpression improves cognitive function and locomotor hyperactivity in an aneuploid mouse model of Down syndrome and its euploid littermates.Acta neuropathologica communications · 2021Article
- Activation of Cilia-Independent Hedgehog/GLI1 Signaling as a Novel Concept for Neuroblastoma Therapy.Cancers · 2021Article
- Sonic Hedgehog Pathway Modulation Normalizes Expression of Olig2 in Rostrally Patterned NPCs With Trisomy 21.Frontiers in cellular neuroscience · 2021Article
- Astrocytes in Down Syndrome Across the Lifespan.Frontiers in cellular neuroscience · 2021Review
- Genetic and epigenetic mechanisms in the development of congenital heart diseases.World journal of pediatric surgery · 2021Review
- Sonic Hedgehog Signaling Agonist (SAG) Triggers BDNF Secretion and Promotes the Maturation of GABAergic Networks in the Postnatal Rat Hippocampus.Frontiers in cellular neuroscience · 2020Article
- Hedgehog Signaling and Embryonic Craniofacial Disorders.Journal of developmental biology · 2019Review
- Down syndrome mouse models have an abnormal enteric nervous system.JCI insight · 2019Article
- Trisomy 21 Represses Cilia Formation and Function.Developmental cell · 2018Article
- Survey of Human Chromosome 21 Gene Expression Effects on Early Development inG3 (Bethesda, Md.) · 2018Article
- Multiple roles of Sonic Hedgehog in the developing human cortex are suggested by its widespread distribution.Brain structure & function · 2018Article
- The Role of Hedgehog Signalling in the Formation of the Ventricular Septum.Journal of developmental biology · 2017Review
- Influence of prenatal EGCG treatment and Dyrk1a dosage reduction on craniofacial features associated with Down syndrome.Human molecular genetics · 2016Article
Corrections and comments
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Authors and funding
3 authors at 2 institutions in 1 country.
Funding
Abstract
The hedgehog (HH) family of growth factors is involved in many aspects of growth and development, from the establishment of left-right axes at gastrulation to the patterning and formation of multiple structures in essentially every tissue, to the maintenance and regulation of stem cell populations in adults. Sonic hedgehog (Shh) in particular acts as a mitogen, regulating proliferation of target cells, a growth factor that triggers differentiation in target populations, and a morphogen causing cells to respond differently based on their positions along a spatial and temporal concentration gradient. Given its very broad range of effects in development, it is not surprising that many of the structures affected by a disruption in Shh signaling are also affected in Down syndrome (DS). However, recent studies have shown that trisomic cerebellar granule cell precursors have a deficit, compared to their euploid counterparts, in their response to the mitogenic effects of Shh. This deficit substantially contributes to the hypocellular cerebellum in mouse models that parallels the human DS phenotype and can be corrected in early development by a single exposure to a small-molecule agonist of the Shh pathway. Here, we consider how an attenuated Shh response might affect several aspects of development to produce multiple phenotypic outcomes observed in DS.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.