DLL1
Homo sapiens
Gene Name: delta like canonical Notch ligand 1
Aliases: DELTA1, DL1, Delta
Chromosome No: 6
Chromosome Band: 6q27
Genetic Category: Rare single gene variant-Syndromic
Aliases: DELTA1, DL1, Delta
Chromosome No: 6
Chromosome Band: 6q27
Genetic Category: Rare single gene variant-Syndromic
Summary Statistics:
ASD Reports: 6
Recent Reports: 1
Annotated variants: 19
Associated CNVs: 5
Evidence score: 3
ASD Reports: 6
Recent Reports: 1
Annotated variants: 19
Associated CNVs: 5
Evidence score: 3
Associated Disorders: |
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Relevance to Autism
A de novo frameshift variant in the DLL1 gene was identified in an ASD proband from the Simons Simplex Collection in O'Roak et al., 2014. Fischer-Zirnsak et al., 2019 identified 15 individuals from 12 unrelated families with heterozygous and likely pathogenic DLL1 variants; the most common phenotypes observed in this cohort were developmental delay/intellectual disability (12/14), autism spectrum disorder (6/14), seizures (6/14), muscular hypotonia (6/14), abnormal brain MRI (11/15), and facial dysmorphism (8/15).
Molecular Function
DLL1 is a human homolog of the Notch Delta ligand and is a member of the delta/serrate/jagged family. It plays a role in mediating cell fate decisions during hematopoiesis. It may play a role in cell-to-cell communication.
References
Type
Title
Type of Disorder
Associated Disorders
Author, Year
Primary
Recurrent de novo mutations implicate novel genes underlying simplex autism risk.
ASD
Support
Mutational Landscape of Autism Spectrum Disorder Brain Tissue
ASD
Recent Recommendation
Haploinsufficiency of the Notch Ligand DLL1 Causes Variable Neurodevelopmental Disorders.
DD, ID
ASD, epilepsy/seizures, hypotonia, abnormal brain
Rare
Variant ID
Variant Type
Allele Change
Residue Change
Inheritance Pattern
Inheritance Association
Family Type
Author, Year
GEN1094R003
stop_gained
c.231C>A
p.Cys77Ter
Familial
Maternal
Multiplex
GEN1094R004
stop_gained
c.1525C>T
p.Arg509Ter
Familial
Paternal
Multi-generational
GEN1094R005
frameshift_variant
c.2013_2014del
p.Glu673GlyfsTer15
Unknown
GEN1094R006
frameshift_variant
c.50_51del
p.Cys17SerfsTer108
Unknown
GEN1094R007
frameshift_variant
c.2013_2014del
p.Glu673GlyfsTer15
De novo
Simplex
GEN1094R008
frameshift_variant
c.1401_1402insCACCT
p.Thr468HisfsTer71
De novo
GEN1094R009
frameshift_variant
c.543_544insACACTACTACGGAGAGGGCTGCTCCGTT
p.His182ThrfsTer59
Unknown
Not maternal
Multi-generational
GEN1094R011
splice_site_variant
c.54_54+1insTAGTCG
p.Gln18_Val19insTer
De novo
Common
No Common Variants Available