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Mendeliome v2.268 FAM210A Achchuthan Shanmugasundram gene: FAM210A was added
gene: FAM210A was added to Mendeliome. Sources: Literature
Mode of inheritance for gene: FAM210A was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: FAM210A were set to 42410297
Phenotypes for gene: FAM210A were set to Spondyloepimetaphyseal dysplasia with tracheal stenosis and ectodermal dysplasia, OMIM:621650
Review for gene: FAM210A was set to GREEN
Added comment: PMID:42410297 (2026) reported five patients from four unrelated families with skeletal dysplasia phenotype characterised by spondyloepimetaphyseal dysplasiachondrodysplasia with short stature (all patents), tracheal stenosis (all patients), conical teeth and/or early tooth decay, and sparse hair suggestive of ectodermal dysplasia (in three unrelated patients).

They were identified with biallelic variants in FAM210A gene (new gene name - MIMS1) - homozygous missense variants in three families and compound heterozygous nonsense variants in the first family with two siblings.

This gene has been associated with relevant phenotype in OMIM (MIM #621650, last accessed 24 July 2026).
Sources: Literature