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11q24.2-q24.3CNV Type: Deletion


Largest CNV size: 2899000 bp

Statistics Box:
Number of Reports: 2



Summary Information

A de novo deletion in the 11q24.2-q24.3 region was detected in a female ASD patient.

Additional Locus Information

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USCS Symbol             NCBI Symbol

Decipher

            Decipher Symbol

References

Major Reports

Title
Author, Year
Report Class
CNV Type
An evidence-based approach to establish the functional and clinical significance of copy number variants in intellectual and developmental disabili...
Deletion
Interstitial deletion of 11q-implicating the KIRREL3 gene in the neurocognitive delay associated with Jacobsen syndrome.
Deletion

Minor Reports

Title
Author, Year
Report Class
CNV Type

No Minor Reports

Cases

Cohort ID
Author, Year
Descripton
Cohort Size
Diagnosis
Age
Gender
CNV Size
Deletion
Duplication
Total CNV's
 guerin_12_ASD_discovery_cases
 Patient referred to clinic (Hospital for Sick Children, Toronto, Canada) with a history of sagittal craniosynostosis, perimembranous ventricular septal defect with patent foramen ovale and autism spectrum disorder (ASD).
 1
 Diagnosis of ASD made at age of 4 years using ADOS (Autism Diagnostic Observation Schedule). Patient also displays clinical manifestations consistent with Jacobsen syndrome.
 4 yrs.
 Female
 2899000
 1
 0
 1
 kaminsky_11_DD/ID/ASD_discovery_cases
 Cases from the International Standards for Cytogenomic Arrays (ISCA) consortium
 15749
 Unexplained developmental delay, intellectual disability, dysmorphic features, multiple congenital anomalies, autism spectrum disorders, or clinical features suggestive of a chromosomal syndrome
 NA
 NA
 3181076
 1
 0
 1

Controls

Cohort ID
Author, Year
Descripton
Cohort Size
Diagnosis
Age
Gender
CNV Size
Deletion
Duplication
Total CNV's
 kaminsky_11_DD/ID/ASD_discovery_controls
 Controls from the International Standards for Cytogenomic Arrays (ISCA) consortium
 10118
 Controls
 NA
 NA
 NA
 NA
 NA
 NA

Cases

Cohort ID
Geographical Ancestry
Discovery Method
Platform
Algorithm
Software
Validation Method
 guerin_12_ASD_discovery_cases
  European
 CMA
 
 
 
 aCGH (Agilent 4x44K)
 kaminsky_11_DD/ID/ASD_discovery_cases
  NA
 aCGH
  Agilent 44K, Agilent 105K
 
 Feature Extraction, DNA Analytics
 FISH, qPCR, MLPA, aCGH, standard G-banded chromosome analysis

Controls

Cohort ID
Geographical Ancestry
Discovery Method
Platform
Algorithm
Software
Validation Method
  kaminsky_11_DD/ID/ASD_discovery_controls
  NA
  aCGH
  Agilent 44K, Agilent 105K
 
  Feature Extraction, DNA Analytics
 

Cases

Patient ID
Author, Year
Age
Gender
Primary Diagnosis
Clinical Profile
Cognitive Profile
CNV Start
CNV End
CNV Size
Genome Build
Type Method
Validation
  guerin_12_ASD_discovery_cases-case1
 4 yrs.
 F
 ASD
 Diagnosis of ASD made at age of 4 years using ADOS. Birth/neonatal history: uncomplicated pregnancy, no abnormalities detected on fetal ultrasounds, negative prenatal screening for Down syndrome and open neural tube defects; delivery was spontaneous, vaginal, at term, and uncomplicated; birth weight 75th %ile; developed respiratory distress at 24 hr of life and was found to have a ventricular septal defect (VSD); sagittal craniosynostosis noted at 1-2 days of life (repaired at 4 months of age). Developmental milestones: early motor milestones reportedly within normal range; significant delays in receptive and expressive langauge and diffculties in social interaction and eye contact became apparent at 2 years of age. Cardiac evaluation: grade 2/6 systolic murmur. Visual and auditory evaluation: normal. Dysmorphic features: facial characteristics consistent with Jacobsen syndrome, including trigonocephaly, hypertelorism with deep-set eyes, prominent supraorbital ridges. Family history: parents were healthy, non-consanguineous, and of European descent.
 
 126897774
 129797236
  2899463
 GRCh38
 Deletion
 Yes
  kaminsky_11_DD/ID/ASD_discovery_cases-ISCA00000921
 NA
 NA
 Developmental delay/intellectual disability/ASD
 Clinical profile NA
 Cognitive profile NA
 125891315
 129072391
  3181077
 GRCh38
 Deletion
 Yes

Controls

No Control Data Available

Cases

Patient ID
Validation Description
Primary Disorder Inheritence
Inheritence
Family Profile
Disease Segregation
Gene Content
Altered Gene Expression
 guerin_12_ASD_discovery_cases-case1
 aCGH (Agilent 4x44K)
 
 De novo
 Simplex
 Likely segregated
 MIR3167,KIRREL3-AS3,RN7SKP121,RN7SKP279,MIR6090,ETS1-AS1,SENCR,C11orf45,RNU6-876P,RNU6-874P,ZNF123P,RPS27P20,KIRREL3-AS2,LINC02098,KCNJ5,TP53AIP1,BARX2,LINC01395,ETS1,FLI1,KCNJ1,KIRREL3,ARHGAP32
 
 kaminsky_11_DD/ID/ASD_discovery_cases-ISCA00000921
 FISH, qPCR, MLPA, aCGH, or standard G-banded chromosome analysis
 
 Unknown
 Unknown
 Unknown
 VSIG10L2,NAP1L1P1,RNU4-86P,RN7SL351P,SRPRA,RPL35AP26,MIR3167,KIRREL3-AS3,RN7SKP121,RN7SKP279,MIR6090,ETS1-AS1,SENCR,C11orf45,RNU6-876P,HYLS1,PUS3,DDX25,RPUSD4,FOXRED1,TIRAP,DCPS,ST3GAL4,KIRREL3-AS1,KIRREL3-AS2,LINC02098,KCNJ5,TP53AIP1,CDON,FAM118B,GSEC,ETS1,FLI1,KCNJ1,KIRREL3,ARHGAP32
 

Controls

No Control Data Available
No Animal Model Data Available
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