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DDG2P v6.8 LBX1 Achchuthan Shanmugasundram Tag founder-effect tag was added to gene: LBX1.
DDG2P v6.8 LBX1 Achchuthan Shanmugasundram commented on gene: LBX1: The DDG2P panel is not curated at Genomics England and is updated only to reflect the latest knowledge from the Gene2Phenotype resource (https://www.ebi.ac.uk/gene2phenotype/). This gene has been added with amber rating on R333 Central congenital hypoventilation panel (https://panelapp.genomicsengland.co.uk/panels/1314/gene/LBX1/) as this phenotype clearly fits into the scope of R333 clinical indication.

The 'curated_removed' tag has been added so that this gene won't be relevant on this panel until it is added to the DD panel on G2P resource.
DDG2P v6.8 LBX1 Achchuthan Shanmugasundram Deleted their comment
DDG2P v6.8 LBX1 Achchuthan Shanmugasundram Publications for gene: LBX1 were set to PMID: 30487221
DDG2P v6.7 LBX1 Achchuthan Shanmugasundram Added comment: Comment on phenotypes: OMIM phenotype accessed on 13 October 2025
DDG2P v6.7 LBX1 Achchuthan Shanmugasundram Phenotypes for gene: LBX1 were changed from to ?Central hypoventilation syndrome, congenital, 3, OMIM:619483; central hypoventilation syndrome, congenital, 3, MONDO:0030539
DDG2P v6.6 LBX1 Achchuthan Shanmugasundram Tag curated_removed tag was added to gene: LBX1.
DDG2P v6.6 LBX1 Achchuthan Shanmugasundram commented on gene: LBX1
DDG2P v6.5 LBX1 Ian Berry gene: LBX1 was added
gene: LBX1 was added to DDG2P. Sources: Expert Review
Mode of inheritance for gene: LBX1 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: LBX1 were set to PMID: 30487221
Penetrance for gene: LBX1 were set to Complete
Review for gene: LBX1 was set to GREEN
gene: LBX1 was marked as current diagnostic
Added comment: Single sib-pair in PMID: 30487221 with congenital central hypoventilation syndrome, 1bp resulting in a frameshift in the terminal exon. Mouse model of the same variant expressed a protein with an altered C-terminal and replicated the human phenotype.

Subsequent unpublished studies (in process of publication): 2 further cases in CVA/GEL dataset with comparable phenotypes, 2 further probands (with segregation) in other families via multinational collaboration.

All cases appear to have the same variant NM_006562.5: c.707del p.(Val236Alafs*59) which may be a Roma founder.
Sources: Expert Review