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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.

External Links

        

References

Type
Title
Type of Disorder
Associated Disorders
Author, Year
Primary
Recurrent de novo mutations implicate novel genes underlying simplex autism risk.
ASD
Support
Integrating de novo and inherited variants in 42
ASD
Support
Mutational Landscape of Autism Spectrum Disorder Brain Tissue
ASD
Support
ASD
DD, ID
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
 GEN1094R001 
 frameshift_variant 
 c.1291dup 
 p.Cys431LeufsTer11 
 De novo 
  
 Simplex 
 GEN1094R002 
 stop_gained 
 c.1492G>T 
 p.Glu498Ter 
 De novo 
  
  
 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 
 GEN1094R010 
 splice_site_variant 
 c.54+1G>A 
  
 De novo 
  
  
 GEN1094R011 
 splice_site_variant 
 c.54_54+1insTAGTCG 
 p.Gln18_Val19insTer 
 De novo 
  
  
 GEN1094R012 
 missense_variant 
 c.536G>T 
 p.Cys179Phe 
 De novo 
  
 Simplex 
 GEN1094R013 
 copy_number_loss 
  
  
 De novo 
  
 Simplex 
 GEN1094R014 
 missense_variant 
 c.503G>C 
 p.Arg168Pro 
 Unknown 
  
  
 GEN1094R015 
 missense_variant 
 c.1957G>A 
 p.Ala653Thr 
 De novo 
  
  
 GEN1094R016 
 synonymous_variant 
 c.1881C>T 
 p.Ala627%3D 
 De novo 
  
  
 GEN1094R017 
 missense_variant 
 c.619C>T 
 p.Arg207Cys 
 De novo 
  
  
 GEN1094R018 
 missense_variant 
 c.422A>G 
 p.Glu141Gly 
 De novo 
  
  
 GEN1094R019 
 missense_variant 
 c.1336G>A 
 p.Asp446Asn 
 Familial 
 Paternal 
 Simplex 

Common

No Common Variants Available
Chromosome
CNV Locus
CNV Type
# of studies
Animal Model
6
Duplication
 1
 
6
Deletion
 3
 
6
Deletion-Duplication
 2
 
6
Deletion
 9
 
6
Deletion-Duplication
 33
 

No Animal Model Data Available

 

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