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T21-eQTL: Dosage Compensation in Down Syndrome

Cohort-scale extension of Hunter et al. (2023) "Transcription dosage compensation does not occur in Down syndrome" (BMC Biology 21:228), applied to the Human Trisome Project (HTP): 304 T21 + 95 Control whole-blood RNA-seq samples, 302 of the T21 subjects with paired chr21 genotypes.

The pipeline asks, for each chromosome 21 protein-coding gene whose ploidy-corrected expression deviates from the 1.5x trisomy expectation by more than typical genome-wide cohort noise: can the deviation be explained by a common cis-acting eQTL in GTEx whole blood that reproduces in our T21 cohort, or does it remain a candidate for true regulatory dosage compensation?

Headline result

Of the 160 chr21 protein-coding genes tested:

Lane Count
Expected dosage (within cohort noise) 119
DE_high, eQTL-supported 6
DE_low, eQTL-supported 9
DE_low, eQTL-tested but unsupported 3 (BACE2, ADARB1, NRIP1)
DE_low, no GTEx cis-eQTL coverage 3 (LTN1, RUNX1, ZBTB21)
High repeats / Low expression / Not DE 20

All higher-than-expected genes (DE_high) are eQTL-supported - acting as a positive control. Among lower-than-expected genes that could be tested against GTEx, 9 of 12 (75%) are eQTL-supported; the remaining 3 (BACE2, ADARB1, NRIP1) plus the 3 untestable ones are the residual compensation candidates.

Quick start

Rscript install_packages.R                        # one-time
Rscript scripts/00_preprocess_data.R              # long -> wide counts
Rscript scripts/01_deseq2_analysis.R              # trisomy-aware DESeq2
Rscript scripts/02_filter_genotypes.R             # gene + variant universe
Rscript scripts/03_t21_dosage_boxplots.R          # within-T21 fits
Rscript scripts/04_chr21_lane_assignment.R        # MAIN per-gene table + SankeyMATIC
Rscript scripts/05_chr21_distribution_panel.R     # population view

The full 00-05 chain runs in ~30 minutes.

AI Assistance

This project utilized the AI assistant Claude, developed by Anthropic, during the development process. Its assistance included generating initial code snippets and improving documentation. All AI-generated content was reviewed, tested, and validated by human developers.

Documentation

See CLAUDE.md for the canonical project documentation: methodology, pipeline walkthrough, data inputs, full script catalog, and output file inventory.

Citation

Hunter, S., Hendrix, J., Freeman, J., Dowell, R.D., & Allen, M.A. (2023). Transcription dosage compensation does not occur in Down syndrome. BMC Biology 21:228. https://doi.org/10.1186/s12915-023-01700-4

License

BSD 3-Clause License — see LICENSE.

About

Cohort-scale extension of Hunter et al. (2023) "Transcription dosage compensation does not occur in Down syndrome" (BMC Biology 21:228), applied to the Human Trisome Project (HTP): 304 T21 + 95 Control whole-blood RNA-seq samples, 302 of the T21 subjects with paired chr21 genotypes.

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