Reelin pathway defects in human type 2 lissencephaly

Exploratory Score: 0.700 Price: $0.50 type 2 lissencephaly human patients Status: proposed

What This Experiment Tests

Exploratory experiment designed to discover new patterns targeting Reelin, lipoprotein receptors, Dab1, integrin alpha 3 beta 1 in human patients. Primary outcome: presence of type 2 lissencephaly and poor cortical lamination

Description

Investigation of human patients with defects in the Reelin signaling pathway, including mutations affecting Reelin itself, lipoprotein receptor family members, the intracellular adaptor Dab1, and possibly integrin alpha 3 beta 1. The study examined how defective Reelin signaling leads to poor cortical lamination and results in a distinct type 2 lissencephaly phenotype, different from type 1 lissencephaly. This research provided insights into the mechanisms of architectonic pattern formation during neuronal migration and the role of recognition-adhesion processes among target neurons.

TARGET GENE
Reelin, lipoprotein receptors, Dab1, integrin alpha 3 beta 1
MODEL SYSTEM
human patients
ESTIMATED COST
$0
TIMELINE
0 months
PATHWAY
Reelin signaling pathway
SOURCE
extracted_from_pmid_11429281
PRIMARY OUTCOME
presence of type 2 lissencephaly and poor cortical lamination

Scoring Dimensions

Info Gain 0.00 (25%) Feasibility 0.00 (20%) Hyp Coverage 0.00 (20%) Cost Effect. 0.00 (15%) Novelty 0.00 (10%) Ethical Safety 0.00 (10%) 0.700 composite

📖 Wiki Pages

Reelin ProteinproteinDAB1 ProteinproteinDAB1 GenegeneNeuronal MigrationmechanismMechanismsindexLissencephalydiseaseviral-post-infectious-alsmechanismEpigenetics in Amyotrophic Lateral SclerosismechanismSleep and Respiratory Network Interaction in ALS —experimentMicrobiome-Gut Barrier Signatures in ALS — ExperimexperimentEnvironmental Exposure Causal Attribution in ALS —experimentAutism Spectrum DisorderdiseaseAmyotrophic Lateral Sclerosis (ALS)diseaseHigh-Dose Vitamins ALS TrialclinicalSodium Phenylbutyrate ALS Trialclinical

Protocol

genetic analysis and neuropathological examination of patients with Reelin pathway defects

Expected Outcomes

defective Reelin signaling would cause poor lamination and type 2 lissencephaly

Success Criteria

demonstration of type 2 lissencephaly phenotype distinct from type 1

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