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Mendeliome v1.3566 NOL10 Zornitza Stark Marked gene: NOL10 as ready
Mendeliome v1.3566 NOL10 Zornitza Stark Gene: nol10 has been classified as Red List (Low Evidence).
Mendeliome v1.3552 NOL10 Sangavi Sivagnanasundram gene: NOL10 was added
gene: NOL10 was added to Mendeliome. Sources: Literature
Mode of inheritance for gene: NOL10 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: NOL10 were set to 41093997
Phenotypes for gene: NOL10 were set to NOL10-related neurological disorder MONDO:0100545
Review for gene: NOL10 was set to RED
Added comment: 12yr F with recurrent focal seizures, progressive memory impairment and atrophy and parietal gliosis on MRI. Homozygous missense variant was identified (NM_024894.4: c.682 A > C; p.Asn228His). The variant is absent in gnomAD v4.1

Functional study using patient-derived fibroblasts was conducted and showed defective ribosome biogenesis. As this is a new gene disease association, it is unclear if that is the mechanism of disease. Need more evidence to promote the gene to Amber.
Sources: Literature