OtherMolecular autism2023

Translatome analysis of tuberous sclerosis complex 1 patient-derived neural progenitor cells reveals rapamycin-dependent and independent alterations.

Inci S Aksoylu, Pauline Martin, Francis Robert and 10 others

PMID 37880800

WHAT IT FOUND

In lab cells from a TSC patient, rapamycin failed to correct neural growth issues.

A newer drug, RMC-6272, reversed these cellular defects and fixed gene translation problems that rapamycin missed.

Key findings

01Rapamycin treatment did not reduce neurite number, length, or extremities in TSC1-null neural progenitor cells.

02The bi-steric mTORC1 inhibitor RMC-6272 significantly reduced neurite number, length, and extremities in TSC1-null cells.

03RMC-6272 reversed a subset of gene translation changes that remained insensitive to rapamycin.

STILL TO COME

How it was doneWhat they found

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What it does not show

The study used cells from only one patient. The postmortem brain analysis included very few samples (6 ASD, 4 controls), limiting statistical power. Findings are from lab cells and postmortem tissue, not living patients. The new drug RMC-6272 was not tested in humans for TSC-related symptoms in this paper.

Declared interests

RMC-6272 was provided by Revolution Medicines, Inc. Funding came from NIH, Wellcome Trust, Swedish Brain Foundation, Swedish Research Council, and Wallenberg Academy Fellow program.

The easy way to misread this

Do not assume RMC-6272 is an available or proven treatment for TSC or autism. This study only tested the drug on cells in a lab dish and on brain tissue after death. It shows the drug works better than rapamycin at fixing specific cellular defects in a dish, but it does not prove it helps patients.

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The study

Certainty of evidence
Low

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    Inci S Aksoylu, Pauline Martin, Francis Robert, et al. Translatome analysis of tuberous sclerosis complex 1 patient-derived neural progenitor cells reveals rapamycin-dependent and independent alterations. Molecular autism. 2023.

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