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1p34.3-p34.2CNV Type: Deletion


Largest CNV size: 3244000 bp

Statistics Box:
Number of Reports: 3



Summary Information

This singleton CNV was identified in an autistic male with mild microcephaly and contains, among other genes, the RIMS3 gene.

Additional Locus Information

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USCS Symbol             NCBI Symbol

Decipher

            Decipher Symbol

References

Major Reports

Title
Author, Year
Report Class
CNV Type
A de novo 1p34.2 microdeletion identifies the synaptic vesicle gene RIMS3 as a novel candidate for autism.
Deletion
An evidence-based approach to establish the functional and clinical significance of copy number variants in intellectual and developmental disabili...
Deletion-Duplication
Both rare and de novo copy number variants are prevalent in agenesis of the corpus callosum but not in cerebellar hypoplasia or polymicrogyria.
Duplication

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
 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
 4218885
 1
 1
 2
 kumar_10_ASD_discovery_cases
 Child with autism and mild microcephaly
 1
 ASD
 10
 M
 3300000
 1
 0
 1
 sajan_13_ACC/CBLH/PMG_discovery_cases
 Individuals with severe congenital brain malformations [agenesis of the corpus callosum (ACC), cerebellar hypoplasia (CBLH), and/or polymicrogyria (PMG)] and additional neurodevelopmental phenotypes
 487
 Diagnosis of agenesis of the corpus callosum (ACC), cerebellar hypoplasia (CBLH), and/or polymicrogyria (PMG); additional diagnoses of autism spectrum disorder (ASD), developmental delay (DD), intellectual disability (ID) and/or seizures in some patients
 N/A
 N/A
 373966
 0
 1
 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
 kumar_10_ASD_discovery_controls
 Control subjects previously used in PMID 18374305 (christian_08_ASD_discovery_controls), from NIMH Genetics Initiative Control sample set, characterized for Axis I disorders
 372
 Controls
 
 
 3300000
 0
 0
 0

Cases

Cohort ID
Geographical Ancestry
Discovery Method
Platform
Algorithm
Software
Validation Method
 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
 kumar_10_ASD_discovery_cases
  Caucasian
 aCGH
  19K whole genome human BAC array, Illumina 610K-Quad
 
 
 FISH
 sajan_13_ACC/CBLH/PMG_discovery_cases
  81.31% Caucasian
 Solid phase hybridization
  Illumina InfiniumII HumanHap610
 PennCNV
 
 qPCR

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
 
  kumar_10_ASD_discovery_controls
  262 Caucasians, 100 African-Maericans
  aCGH
  19K whole genome human BAC array, Illumina 610K-Quad
 
 
 

Cases

Patient ID
Author, Year
Age
Gender
Primary Diagnosis
Clinical Profile
Cognitive Profile
CNV Start
CNV End
CNV Size
Genome Build
Type Method
Validation
  kaminsky_11_DD/ID/ASD_discovery_cases-ISCA00001175
 NA
 NA
 Developmental delay/intellectual disability/ASD
 Clinical profile NA
 Cognitive profile NA
 38108665
 42327551
  4218887
 GRCh38
 Deletion
 Yes
  kaminsky_11_DD/ID/ASD_discovery_cases-ISCA00005417
 NA
 NA
 Developmental delay/intellectual disability/ASD
 Clinical profile NA
 Cognitive profile NA
 39360747
 40900817
  1540071
 GRCh38
 Duplication
 Yes
  kumar_10_ASD_discovery_cases-patientLP99-105
 10
 M
 ASD
 Mild microcephaly, poor language and social skills, repetitive behaviors
 NA
 39556037
 42820716
  3264680
 GRCh38
 Deletion
 Yes
  sajan_13_ACC/CBLH/PMG_discovery_cases-case1308-0
 N/A
 N/A
 ACC
 Diagnosis of agenesis of the corpus callosum (ACC). ASD: yes. Seizures: no.
 Developmental delay: yes. Intellectual disability: yes.
 39079597
 39453562
  373966
 GRCh38
 Duplication
 Yes

Controls

No Control Data Available

Cases

Patient ID
Validation Description
Primary Disorder Inheritence
Inheritence
Family Profile
Disease Segregation
Gene Content
Altered Gene Expression
 kaminsky_11_DD/ID/ASD_discovery_cases-ISCA00001175
 FISH, qPCR, MLPA, aCGH, or standard G-banded chromosome analysis
 
 Unknown
 Unknown
 Unknown
 LINC01343,RNU6-753P,MYCBP,RNU6-605P,EIF1P2,RNU6-608P,RNA5SP44,HSPE1P8,OXCT2P1,SNORA55,RNU7-121P,OXCT2,MYCL,RNU6-1237P,TMCO2,EXO5,GTF2F2P2,NFYC-AS1,MIR30E,MIR30C1,RN7SL326P,UBE2V1P8,SLFNL1,RPL23AP17,RNA5SP45,GUCA2B,GUCA2A,LINC01685,RRAGC,GJA9-MYCBP,GJA9,AKIRIN1,NDUFS5,BMP8A,PPIEL,PABPC4,HEYL,NT5C1A,HPCAL4,BMP8B,MFSD2A,CAP1,PPT1,ZMPSTE24,COL9A2,ZFP69B,ZFP69,ZNF684,KCNQ4,CITED4,CTPS1,FOXO6,EDN2,HNRNPFP1,RHBDL2,MACF1,PPIE,RLF,SMAP2,RIMS3,NFYC,SLFNL1-AS1,SCMH1,FOXJ3,TRIT1,HIVEP3
 
 kaminsky_11_DD/ID/ASD_discovery_cases-ISCA00005417
 FISH, qPCR, MLPA, aCGH, or standard G-banded chromosome analysis
 
 Unknown
 Unknown
 Unknown
 OXCT2P1,SNORA55,RNU7-121P,OXCT2,MYCL,RNU6-1237P,TMCO2,EXO5,GTF2F2P2,NFYC-AS1,MIR30E,MIR30C1,RN7SL326P,BMP8A,PPIEL,PABPC4,HEYL,NT5C1A,HPCAL4,BMP8B,MFSD2A,CAP1,PPT1,ZMPSTE24,COL9A2,ZFP69B,ZFP69,ZNF684,KCNQ4,CITED4,MACF1,PPIE,RLF,SMAP2,RIMS3,NFYC,TRIT1
 
 kumar_10_ASD_discovery_cases-patientLP99-105
 FISH
 
 De novo
 NA
 NA
 SNORA55,RNU7-121P,OXCT2,MYCL,RNU6-1237P,TMCO2,EXO5,GTF2F2P2,NFYC-AS1,MIR30E,MIR30C1,RN7SL326P,UBE2V1P8,SLFNL1,RPL23AP17,RNA5SP45,GUCA2B,GUCA2A,TMSB4XP1,RNU6-536P,CLDN19,PPIEL,PABPC4,HEYL,NT5C1A,HPCAL4,BMP8B,MFSD2A,CAP1,PPT1,ZMPSTE24,COL9A2,ZFP69B,ZFP69,ZNF684,KCNQ4,CITED4,CTPS1,FOXO6,EDN2,HNRNPFP1,RIMKLA,ZMYND12,PPIH,YBX1,P3H1,C1orf50,TMEM269,SVBP,ERMAP,PPIE,RLF,SMAP2,RIMS3,NFYC,SLFNL1-AS1,SCMH1,FOXJ3,PPCS,CCDC30,TRIT1,HIVEP3
 
 sajan_13_ACC/CBLH/PMG_discovery_cases-case1308-0
 qPCR
 
 Unknown
 Unknown
 Unknown
 RNU6-608P,RNA5SP44,HSPE1P8,MACF1
 

Controls

No Control Data Available
No Animal Model Data Available
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