Length (hg19) : 594,340 bases - Length (hg38) : 374,197 bases


skin skin skin skin skin skin skin skin skin skin skin skin skin skin skin

CNV-Hub AChro-Puce
Benign



AChro-Puce Criteria taken into account 1

Class

1 

At least 3 occurrences in DGV / DGV-Gold > 80 % overlaps

Class

2 Major

CNV inherited from Asymptomatic parent

Class

2 Major

At least 1 occurrence in DGV / DGV-Gold > 80 % overlaps

Class

2 Minor

CNV more frequent in Coe & Al controls than in patient cases

Class

2 Minor

At least 1 occurrence in DGV / DGV-Gold > 50 % overlaps

Class

4 Major

De novo CNV or inherited from Symptomatic parent


ISV 2

XCNV 3

ClassifyCNV ACMG 4

AnnotSV ACMG 5

ACMG criteria

ClassifyCNV

4O
-1

Overlap with common population variation.

5B
-0.45

Patient with specific, well-defined phenotype and no family history. CNV is inherited from an apparently unaffected parent.

5D
+ 0.45

CNV segregates with a consistent phenotype observed in the patient’s family.

AnnotSV

4O
-1

Overlap with common population variation.

5B
-0.45

Patient with specific, well-defined phenotype and no family history. CNV is inherited from an apparently unaffected parent.

5D
+ 0.45

CNV segregates with a consistent phenotype observed in the patient’s family.


ClinGen

7 benign CNV
0 likely benign CNV
0 uncertain CNV
0 likely pathogenic CNV
0 pathogenic CNV

70% Overlaps


Decipher

0 benign CNV
0 unknown CNV
0 uncertain CNV
0 pathogenic CNV

70% Overlaps

DGV-Gold

1

80% Overlaps

0

50% Overlaps


DGV

4

80% Overlaps

17

50% Overlaps


Coe & Al study 6

1

Patient cases
70% Overlaps

22

Controls
70% Overlaps


Genes with pHaplo > 0.55 7

0

Genes with pTriplo > 0.68 7

0

Genes in SFARI

0

Genes in OMIM

2


Sources and references

1 : AChroPuce Consortium Recommandations pour l’interpretation Clinique des CNV (Copy Number Variations) Septembre 2022.

2 : Automated prediction of the clinical impact of structural copy number variations : M. Gažiová, T. Sládeček, O. Pös, M. Števko, W. Krampl, Z. Pös, R. Hekel, M. Hlavačka, M. Kucharík, J. Radvánszky, J. Budiš & T. Szemes View article

3 : Zhang L, Shi J, Ouyang J, Zhang R, Tao Y, Yuan D, et al X CNV genome wide prediction of the pathogenicity of copy number variations Genome Med 2021 13 132.

4 : Gurbich, T.A., Ilinsky, V.V. ClassifyCNV: a tool for clinical annotation of copy-number variants. Sci Rep 10, 20375 (2020). View article

5 : Geoffroy V, Herenger Y, Kress A, et al. AnnotSV: an integrated tool for structural variations annotation. Bioinforma Oxf Engl. 2018;34(20):3572-3574. doi:10.1093/bioinformatics/bty304

6 : Coe BP, Witherspoon K, Rosenfeld JA, van Bon BWM, Vulto van Silfhout AT, Bosco P, et al Refining analyses of copy number variation identifies specific genes associated with developmental delay Nat Genet 2014 46 1063 71

7 : Collins RL, Glessner JT, Porcu E, Lepamets M, Brandon R, Lauricella C, et al A cross disorder dosage sensitivity map of the human genome Cell 2022 185 3041 3055 e 25

Delete and Recompute CNV

0 Microdeletion and microduplication syndromes from litterature (>= 70% only)


2 OMIM Gene overlap(s)

Download genes as .csv

GPRIN2 NM_001385282.1   Exons 1->3 / 3 - Size : 14,922 bases


pLI : 0 LOEUF : 1.49 sHet : 0.004 pHaplo : 0.12 pTriplo : 0.29
Location : 46,992,959 - 47,007,881

Database :

DecipherGenomics OMIM:611240 GTEx Portal Human Protein Atlas Ensembl

NPY4R NM_005972.6   Exons 1->3 / 3 - Size : 4,863 bases


pLI : 0 LOEUF : 1.75 sHet : 0.004 pHaplo : 0.14 pTriplo : 0.25
Location : 47,083,457 - 47,088,320

Database :

DecipherGenomics OMIM:601790 GTEx Portal Human Protein Atlas Ensembl


18 Non-OMIM Gene overlap(s)

NOVEL
Size : 162,265 bases


Location : 47,011,753 - 47,174,018

KNOWN
Size : 47,894 bases


Location : 47,191,844 - 47,239,738

KNOWN
Size : 40,985 bases


Location : 47,379,727 - 47,420,712

NOVEL
Size : 35,697 bases


Location : 47,207,805 - 47,243,502

KNOWN
Size : 16,110 bases


Location : 47,157,983 - 47,174,093

KNOWN
Size : 13,576 bases


Location : 47,228,366 - 47,241,942

NOVEL
Size : 10,760 bases


Location : 47,279,073 - 47,289,833

NPY4R2
Size : 4,863 bases


Location : 47,083,457 - 47,088,320

NOVEL
Size : 5,982 bases


Location : 47,186,203 - 47,192,185

ENSG00000273760
Size : 3,451 bases


Location : 47,135,135 - 47,138,586

KNOWN
Size : 4,477 bases


Location : 47,177,204 - 47,181,681

KNOWN
Size : 1,760 bases


Location : 47,254,247 - 47,256,007

HNRNPA1P33
Size : 560 bases


Location : 47,133,338 - 47,133,898

KNOWN
Size : 297 bases


Location : 47,174,442 - 47,174,739

KNOWN
Size : 115 bases


Location : 47,222,341 - 47,222,456

KNOWN
Size : 1,120 bases


Location : 47,271,222 - 47,272,342

KNOWN
Size : 283 bases


Location : 47,382,464 - 47,382,747

KNOWN
Size : 599 bases


Location : 47,396,223 - 47,396,822

7 ClinGen CNV overlap(s) (>= 70% only)

7 Benign CNV    0 Likely benign CNV    0 Uncertain CNV    0 Likely pathogenic CNV    0 Pathogenic CNV

#1 Benign (nssv706847)
Location : 46,984,912 - 47,590,979 | Size : 606,067 bases

Score : 0

Mean Coverage : 98 %


#2 Benign (nssv579542)
Location : 47,006,953 - 47,531,169 | Size : 524,216 bases

Score : 1

Mean Coverage : 94 %


#3 Benign (nssv575272)
Location : 46,984,912 - 47,655,146 | Size : 670,234 bases

Score : 0

Mean Coverage : 93 %


#4 Benign (nssv1603316)
Location : 46,951,228 - 47,768,540 | Size : 817,312 bases

Score : 0

Mean Coverage : 84 %


#5 Benign (nssv706710)
Location : 46,984,912 - 47,410,481 | Size : 425,569 bases

Score : 0

Mean Coverage : 83 %


#6 Benign (nssv584008)
Location : 46,951,228 - 47,410,481 | Size : 459,253 bases

Score : 0

Mean Coverage : 82 %


#7 Benign (nssv3397460)
Location : 47,006,953 - 47,323,674 | Size : 316,721 bases

Score : 0

Mean Coverage : 70 %



0 Decipher CNV overlap(s) (>= 70% only)

0 Benign CNV    0 Unknown CNV    0 Uncertain CNV    0 Pathogenic CNV


0 Gene(s) in SFARI Database


4 DGV-Gold overlap(s) (>= 50% only)

DGV-Gold #1
Location : 46,794,787 - 47,748,159 | Size : 953,372 bases

Frequency in Population : 0.26 %
Inner Rank : 12

Mean Coverage : 77 %


DGV-Gold #2
Location : 46,998,307 - 47,587,348 | Size : 589,041 bases

Frequency in Population : 0.34 %
Inner Rank : 12

Mean Coverage : 97 %


DGV-Gold #3
Location : 47,036,780 - 47,311,536 | Size : 274,756 bases

Frequency in Population : 0.58 %
Inner Rank : 16

Mean Coverage : 63 %


DGV-Gold #4
Location : 47,236,543 - 47,461,104 | Size : 224,561 bases

Frequency in Population : 0.24 %
Inner Rank : 7

Mean Coverage : 55 %



17 DGV overlap(s) (>= 50% only)

DGV #1
Location : 46,045,761 - 47,593,951 | Size : 1,548,190 bases

Mean Coverage : 55 %


DGV #2
Location : 46,478,317 - 47,429,141 | Size : 950,824 bases

Mean Coverage : 59 %


DGV #3
Location : 46,590,219 - 47,392,320 | Size : 802,101 bases

Mean Coverage : 60 %


DGV #4
Location : 46,674,168 - 47,652,803 | Size : 978,635 bases

Mean Coverage : 76 %


DGV #5
Location : 46,674,168 - 47,703,613 | Size : 1,029,445 bases

Mean Coverage : 73 %


DGV #6
Location : 46,698,486 - 47,330,067 | Size : 631,581 bases

Mean Coverage : 58 %


DGV #7
Location : 46,918,172 - 47,249,575 | Size : 331,403 bases

Mean Coverage : 59 %


DGV #8
Location : 46,918,172 - 47,392,320 | Size : 474,148 bases

Mean Coverage : 78 %


DGV #9
Location : 46,943,377 - 47,249,575 | Size : 306,198 bases

Mean Coverage : 61 %


DGV #10
Location : 46,943,377 - 47,742,094 | Size : 798,717 bases

Mean Coverage : 85 %


DGV #11
Location : 46,961,233 - 47,743,504 | Size : 782,271 bases

Mean Coverage : 86 %


DGV #12
Location : 46,961,423 - 47,392,320 | Size : 430,897 bases

Mean Coverage : 81 %


DGV #13
Location : 47,003,692 - 47,743,504 | Size : 739,812 bases

Mean Coverage : 85 %


DGV #14
Location : 47,025,092 - 47,392,320 | Size : 367,228 bases

Mean Coverage : 76 %


DGV #15
Location : 47,029,572 - 47,392,320 | Size : 362,748 bases

Mean Coverage : 76 %


DGV #16
Location : 47,035,615 - 47,249,575 | Size : 213,960 bases

Mean Coverage : 53 %


DGV #17
Location : 47,063,105 - 48,174,779 | Size : 1,111,674 bases

Mean Coverage : 59 %



1 Patient cases (>= 70% only)

47,006,953 - 47,531,169
Size : 524,216 bases
11 Reports

Mean Coverage : 94 %


22 Controls (>= 70% only)

nssv3510229
Location : 46674167 - 47703613 | Size : 1029446 bases

Mean Coverage : 73 %


nssv3509892
Location : 46674167 - 47531169 | Size : 857002 bases

Mean Coverage : 76 %


nssv3504648
Location : 46691158 - 47531169 | Size : 840011 bases

Mean Coverage : 77 %


29 Reports
nssv3709060 nssv3707895 nssv3705767 nssv3705763 nssv3705749 nssv3705738 nssv3705733 nssv3521681 nssv3518649 nssv3516097 nssv3514926 nssv3514905 nssv3514847 nssv3514072 nssv3514000 nssv3513409 nssv3513378 nssv3510107 nssv3510089 nssv3509582 nssv3508455 nssv3508019 nssv3507269 nssv3506922 nssv3505974 nssv3505251 nssv3504099 nssv3503873 nssv3502829

Location : 47006953 - 47323674 | Size : 316721 bases

Mean Coverage : 70 %


nssv3705769
Location : 47006953 - 47704625 | Size : 697672 bases

Mean Coverage : 87 %


2 Reports
nssv3709061 nssv3512159

Location : 47006953 - 47531169 | Size : 524216 bases

Mean Coverage : 94 %


2 Reports
nssv3705875 nssv3705874

Location : 47062298 - 47531169 | Size : 468871 bases

Mean Coverage : 88 %


nssv3517824
Location : 47068214 - 47531169 | Size : 462955 bases

Mean Coverage : 88 %


nssv3512828
Location : 47076674 - 47703613 | Size : 626939 bases

Mean Coverage : 81 %


nssv3511103
Location : 47076674 - 47703282 | Size : 626608 bases

Mean Coverage : 81 %


nssv3515011
Location : 47080229 - 47531169 | Size : 450940 bases

Mean Coverage : 86 %


nssv3505128
Location : 47084772 - 47703613 | Size : 618841 bases

Mean Coverage : 80 %


nssv3519352
Location : 47085302 - 47743504 | Size : 658202 bases

Mean Coverage : 77 %


nssv3518624
Location : 47090538 - 47742076 | Size : 651538 bases

Mean Coverage : 77 %


nssv3515171
Location : 47096229 - 47743504 | Size : 647275 bases

Mean Coverage : 76 %


nssv3514303
Location : 47112546 - 47703282 | Size : 590736 bases

Mean Coverage : 77 %


nssv3515285
Location : 47117348 - 47531169 | Size : 413821 bases

Mean Coverage : 82 %


nssv3518831
Location : 47119684 - 47531169 | Size : 411485 bases

Mean Coverage : 82 %


nssv3515289
Location : 47121368 - 47743504 | Size : 622136 bases

Mean Coverage : 74 %


nssv3513817
Location : 47129683 - 47742076 | Size : 612393 bases

Mean Coverage : 73 %


nssv3502876
Location : 47132140 - 47531169 | Size : 399029 bases

Mean Coverage : 80 %


nssv3514906
Location : 47136995 - 47743504 | Size : 606509 bases

Mean Coverage : 72 %



0 Gene(s) in PanelApp Database



ClassifyCNV ACMG Score

Benign

ClassifyCNV ACMG Criteria

4O
-1

Overlap with common population variation.

5B
-0.45

Patient with specific, well-defined phenotype and no family history. CNV is inherited from an apparently unaffected parent.

5D
+ 0.45

CNV segregates with a consistent phenotype observed in the patient’s family.

AnnotSV Score

Benign

AnnotSV ACMG Criteria

4O
-1

Overlap with common population variation.

5B
-0.45

Patient with specific, well-defined phenotype and no family history. CNV is inherited from an apparently unaffected parent.

5D
+ 0.45

CNV segregates with a consistent phenotype observed in the patient’s family.