Foundational

Original disease description and authoritative gene-level resources.

American Journal of Human Genetics · 2019

Haploinsufficiency of the Notch Ligand DLL1 Causes Variable Neurodevelopmental Disorders

Fischer-Zirnsak B, Segebrecht L, Schubach M, et al.

First description of NEDBAS: 15 individuals from 12 families with heterozygous DLL1 variants — established the dominant haploinsufficiency mechanism and the OMIM #618709 phenotype entry.

GenCC / ClinGen · 2023

ClinGen Gene-Disease Validity: DLL1 — NEDBAS, Definitive (Autosomal Dominant)

ClinGen Brain Malformation GCEP

Independent expert review classified the DLL1–NEDBAS association as Definitive — the highest evidence tier for a gene-disease relationship.

OMIM · 2024

DLL1 — Delta-Like Canonical Notch Ligand 1 (gene entry)

OMIM curators

Canonical gene entry: 6q27, 11 exons, encodes a 723-aa type-I transmembrane DSL-family ligand for NOTCH receptors.

Mechanism & functional studies

Animal models and molecular work supporting Notch-pathway disruption.

Frontiers in Neuroscience · 2022

Dll1 haploinsufficiency causes brain abnormalities with functional relevance

Aguilar-Hernández L, et al.

Mouse model showing Dll1+/- animals develop the brain abnormalities and behavioural phenotypes seen in patients — provides the first functional evidence supporting haploinsufficiency.

SCDO7 (recessive form)

Vertebral segmentation literature relevant to the rare recessive form.

OMIM · 2024

Spondylocostal Dysostosis 7 (SCDO7), Autosomal Recessive — OMIM entry

OMIM curators

Catalog entry establishing SCDO7 as a distinct autosomal recessive condition caused by biallelic DLL1 missense variants — to date documented in a single consanguineous family.

GeneReviews® (NCBI Bookshelf) · 2023

Spondylocostal Dysostosis, Autosomal Recessive — GeneReviews

Turnpenny PD, Sloman M, Dunwoodie S

Authoritative clinical synopsis of recessive SCDO including DLL3, MESP2, LFNG, HES7, TBX6, RIPPLY2, and DLL1 (SCDO7) — diagnostic criteria, surveillance, and management.

Frontiers in Genetics · 2022

Clinical genetics of spondylocostal dysostosis: A mini review

Umair M, Younus M, Shafiq S, Nayab A, Alfadhel M

Concise review covering all known SCDO subtypes including the DLL1-associated SCDO7 — useful primer for clinicians evaluating vertebral segmentation defects.

6q27 terminal deletions

Cytogenetic and prenatal series where DLL1 is part of a larger deletion.

medRxiv (preprint, later peer-reviewed) · 2022

The phenotypic spectrum of terminal 6q deletions based on a large cohort derived from social media and literature: a prominent role for DLL1

Engwerda A, et al.

Largest cohort to date of terminal 6q deletions — demonstrates that DLL1 loss is a major driver of neurodevelopmental features regardless of additional co-deleted genes.

Frontiers in Genetics · 2025

DLL1 haploinsufficiency in prenatal brain anomalies: a retrospective analysis of 6q terminal deletions

Multiple authors

Retrospective analysis linking DLL1 haploinsufficiency to specific prenatal brain findings (cerebellar hypoplasia, ventriculomegaly) detectable on fetal imaging.

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