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Garvie, A.

Publications and source records attributed to Garvie, A..

2 recordsLinked to original sources

Integrated analysis of ribosomal DNA copy number and methylation using nanopore long-read sequencing

Ribosomal RNA (rRNA) provides the structural and catalytic core of ribosomes and is encoded by ribosomal RNA genes (rDNA) arranged in tandem repeat arrays. rDNA copy number (CN) is highly dynamic, representing a clinically relevant form of structural variation, but its accurate quantification has been challenging due to its highly repetitive and GC-rich nature. Here, we present RICO (Ribosomal DNA Integrated Copy Number and Methylation Analysis), a novel computational pipeline for integrated estimation of rDNA CN and methylation using nanopore long-read sequencing. RICO leverages long sequencing reads that span entire rDNA repeats, mapped to an rDNA-augmented reference genome, and normalizes coverage using an array of single-copy genes. We show that RICO provides accurate rDNA CN estimates in simulated datasets and reproducible measurements across human samples, with strong agreement to short-read sequencing and PCR-based methods. As biological validation, RICO detects a [~]40% reduction in rDNA CN in Atrx-knockout mouse cells, consistent with established effects of ATRX loss on rDNA CN, and captures detected increased total and active rDNA CN in malignant cells from a MYC-driven B-cell lymphoma mouse model, in line with prior psoralen-based chromatin studies. Applying RICO to independent human cohorts, we uncover that individuals with higher total rDNA CN consistently exhibited higher fractions of high-methylated rDNA copies, suggesting a dosage compensation mechanism that potentially maintains a similar number of active rDNA copies across individuals. Together, RICO enables integrated analysis of rDNA CN and methylation state, providing a scalable framework for investigating rDNA regulation across population and disease studies.

bioinformatics↗

Y chromosome damage underlies testicular abnormalities in ATR-X syndrome

ATR-X (alpha thalassemia, mental retardation, X-linked) syndrome is a severe developmental disorder affecting males caused by mutations in the chromatin remodelling gene ATRX. Genital abnormalities in affected boys include hypospadias and ambiguous genitalia, and patients show small poorly formed testes with only a few seminiferous tubules. Our mouse model recapitulated these testicular defects when Atrx was specifically deleted in Sertoli cells (ScAtrxKO). ScAtrxKO mice develop small testes with fewer and discontinuous tubules due to G2/M arrest and apoptosis of Sertoli cells. Here, we investigated the mechanism underlying the Sertoli cell defects in ATR-X syndrome. In healthy male control mice, Sertoli cell nuclei contain a single novel "GATA4 PML nuclear body (NB)" that strongly expresses the transcription factor GATA4, as well as ATRX and its binding partner DAXX. The GATA4 PML NB co-localizes with heterochromatin protein HP1 and PH3 (a marker of chromosome condensation), and with the short arm of the Y chromosome (Yp). In contrast, ScAtrxKO Sertoli cells contain a single giant GATA4 PML NB, frequently associated with DNA double-strand breaks in G2/M-arrested Sertoli cells that underwent apoptosis. HP1 and PH3 were absent from the giant GATA4 foci suggesting a local failure in heterochromatin formation and chromosome condensation. Our data indicate that in Sertoli cells, ATRX protects a chromosomal region of Yp from DNA damage, probably during replication stress, and thus protects Sertoli cells from cell death. We discuss Y chromosome damage as a novel mechanism for testicular failure and the potential role of GATA4 during this process. Disclosure SummaryThe authors have nothing to disclose.

developmental biology↗