PAFAH1B1/LIS1 mutations in type 1 lissencephaly

Exploratory Score: 0.800 Price: $0.50 type 1 lissencephaly human patients Status: proposed

What This Experiment Tests

Exploratory experiment designed to discover new patterns targeting PAFAH1B1 (LIS1) in human patients. Primary outcome: presence of type 1 lissencephaly

Description

Investigation of human patients with mutations in the PAFAH1B1 gene (LIS1) that cause type 1 lissencephaly. The study examined how LIS1 protein, which interacts with microtubules and is critical for nucleokinesis (nuclear translocation during neuronal migration), when mutated leads to severe brain malformation characterized by smooth brain surface due to defective neuronal migration. This provides insight into the microtubule-dependent mechanisms of neuronal migration.

TARGET GENE
PAFAH1B1 (LIS1)
MODEL SYSTEM
human patients
ESTIMATED COST
$0
TIMELINE
0 months
PATHWAY
microtubule network/nucleokinesis
SOURCE
extracted_from_pmid_11429281
PRIMARY OUTCOME
presence of type 1 lissencephaly

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%) 0.800 composite

📖 Wiki Pages

LIS1 ProteinproteinPAFAH1B1 — Platelet Activating Factor AcetylhydrolgeneLIS1 GenegeneNeuronal MigrationmechanismMechanismsindexLissencephalydisease

Protocol

genetic analysis and neuropathological examination of patients with PAFAH1B1 mutations

Expected Outcomes

mutations in LIS1 would cause defective nucleokinesis leading to type 1 lissencephaly

Success Criteria

demonstration of type 1 lissencephaly phenotype in patients with LIS1 mutations

Related Hypotheses (0)

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