James Fasham
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jamesfasham.bsky.social
James Fasham
@jamesfasham.bsky.social
🧬👨‍⚕️Academic Consultant in Clinical Genetics 💬 ESHG Social media chair. 🤖 @DiseaseGenes bot creator #Genetics #Genomics #RareDisease
Amicia Phillips challenging traditional definitions of 'actionability' in the context of genomic #newbornscreening #ICoNS25
October 24, 2025 at 1:30 PM
Prof. Caroline Wright @UniofExeter explaining the challenges of quantifying penetrance of rare diseases in population cohorts at @ICoNSeqOrg #ICoNSs25

Beware ⚠️
- accidental inclusion of benign variants
- relying on limited phenotype data / codes which may be erroneous
October 24, 2025 at 11:03 AM
Alistair Pagnamenta: rlReduced OI penetrance in @uk_biobank, implications for genomic newborn screening at #ICoNS25.

🦴 majority with P/LP variants have do not have multiple fractures

Impact on protein supported by proteomic data

👀 Preprint out today
www.medrxiv.org/content/10.1...
October 24, 2025 at 10:46 AM
At #ICoNS25 meeting in London the last 2 days (sorry for absence of livetweets!)

Exploring the future of genomics as part of routine #NewbornScreening worldwide
October 24, 2025 at 10:34 AM
Mathilde DOYARD #ESHG2025

Starting with information that TBXAS1 disorders ⬆️ bone density

💊Could it be a target to treat bone fragility disorders such as OI?

thromboxane synthase (TXAS) inhibitors
in 🐭 studies
⬆️ bone formation
⬆️ biomechanical profiles

Patients next?
May 27, 2025 at 10:27 AM
Edu Calpena #ESHG2025
@educalpena.bsky.social

Ectopic SIX3 expression underlies the craniofacial syndrome caused by deletions at the SIX2 locus

"Ectopic expression of SIX3 in the SIX2 territory"

2p21 deletions with TAD disruption shown below
May 27, 2025 at 10:10 AM
Hristiana Lyubenova #ESHG2025

The absence of an enzyme-rescue metabolite as the cause of Catel-Manzke syndrome

A deep dive into uncovering the mechanism of this well-recognised condition
May 27, 2025 at 9:48 AM
Eike Bolmer #ESHG2025

🩻 Bone2Gene-Screen: AI-Augmented Early Detection of Rare Bone Diseases

✋Using hand x-ray

🔴 Occlusion testing allows important region within the image to be defined (see below)
May 27, 2025 at 9:33 AM
Chofit Chai #ESHG2025

Loss of CELSR1 and impaired Wnt/planar cell polarity signaling cause yellow nail syndrome
May 27, 2025 at 9:24 AM
Vicente Yepez #ESHG2025

Rare Disease multi-OMICs Solvathons - a disease solving hackathon
- make sure you have consent to share

Integrated multi-omics in parallel is best

DNA-RNA & DNA-proteomic parallel approaches both ⬆️ diagnoses 10-15% (many refs)
May 27, 2025 at 8:05 AM
Kym Boycott #ESHG2025

Disease-Gene discoveries are falling 😲

Don't tell @DiseaseGenes bot! 🤖
#MorbidGene

In all seriousness, I suspect that conditions associated with each gene are growing - we are in a phase of understanding of gene pleiotropy
May 27, 2025 at 7:23 AM
Kym Boycott #ESHG2025

What is matchmaking
One? Two? Zero? sided

Why do we do it (see the photo below ☺️)

👍👍@deciphergenomics.bsky.social second largest contributor to MatchMaker Exchange

❓sadly, 94% of genematcher entries have no phenotype DECIPHER is much better
May 27, 2025 at 7:17 AM
Mirjam Plantinga #ESHG2025
Ethical, Legal and Social Implications of reanalysis - a review

❓What does reanalysis mean? (See slide)
Differentiate
- Reanalysis, Reinterpretation, Reclassification
Reanalysis of what?
Cost effective?
May 26, 2025 at 3:58 PM
Zornitza Stark
@zornitza.bsky.social
#ESGH2025

🤖 TALOS automatically and iteratively re-reviews genomes and exomes (including CNVs/SV on historic data)

focus on specificity - only 0.7 candidates /trio

Diagnostic yield is 5%, 248/4,744
- higher in older cases 9% 2019 4% 2022
May 26, 2025 at 3:45 PM
The band are warming up for tonight! 🎸🎶 #ESHG2025
May 26, 2025 at 2:40 PM
Nicola Whiffin @nickywhiffin.bsky.social #ESHG2025

Saturation genome editing of RNU4-2 reveals distinct dominant and recessive neurodevelopmental disorders

Saturation mutagenesis outperforms CADD

Novel *AR* NDD with neuroradiological features (below) - ~30 families #MorbidGene
May 26, 2025 at 9:45 AM
Kartik Chundru (Exeter) @Chundru.bsky.social

Comprehensive characterisation of non-coding and coding effects of de novo mutations in a large-scale rare disease case-control cohort

Trios from GEL, UKB & AllOfUs

Highlight RNU non coding variants & splice site

#ESHG2025
May 26, 2025 at 9:37 AM
Looking for a workshop at 14:15? #ESHG2025
Since you're currently reading this, how about...

"Has social media broken scientific knowledge sharing?"
- subject experts, lively debate, audience engagement

📍 Sequencing Square (↗️ turn right as you enter exhhibitor hall)
May 26, 2025 at 9:28 AM
Sébastien Küry #ESHG2025

Unveiling the crucial neuronal role of the proteasomal ATPase subunit gene PSMC5 in neurodevelopmental proteasomopathies
May 26, 2025 at 9:06 AM
Rob Harkness #ESHG2025

Genetic risk of acute-onset axonal neuropathy following infection

RCC1 is novel AR #DiseaseGene

Onset ~18m #GuillianBarre-like
Clinical course #ALS-like

Proteins reduced thermal stability
🪰 reduced survival to paraquat stress

papers.ssrn.com/sol3/papers....
May 26, 2025 at 8:58 AM
⛓️‍💥Late breaking abstracts - always a great session - Space 3 #ESHG2025

Zohreh Fattahi

A new role of the deubiquitinase CYLD for mitochondrial function and integrity- promising therapeutic impact for the treatment of neurodegenerative diseases
May 26, 2025 at 8:45 AM
Next up in #gNBS at #ESHG2025
Wendy Chung (Guardian study)

Variant-Phenotype(-Penetrance) need to be considered. Gene-Disease too simplistic

SCN1A challenging, often inherited

Recessive disease is tricky (in trans?) gnomAD concurrence is helpful
May 26, 2025 at 7:08 AM
@zornitza.bsky.social opening a well-attended Newborn Screening session with a talk on choosing conditions

Subjectivity in application of widely agreed inclusion criteria leads to small overlap (55 genes)

pubmed.ncbi.nlm.nih.gov/38275146/
May 26, 2025 at 6:43 AM
Looking for a poster?

Have a look at P14 002.B and then message me! I would love to discuss!

🧠 New degenerative neurological condition
4️⃣ Caused by a triplication on 16p
🔁 Mediated by a palindromic repeat

#ESHG2025
May 25, 2025 at 3:53 PM
Siddharth Banka #ESHG2025

R-loop forming regions genes #RNU2-2 and #RNU5B-1 novel non-coding NDD genes #MorbidGene

Clinical features of these new "RNU"pathies
- Hypotonia, macrocephaly, seizures, FTT
- explain 10-15 / 1000 rare NDDs

www.medrxiv.org/content/10.1...
May 25, 2025 at 9:49 AM