Unique variants in the FBN2 gene

Information The variants shown are described using the NM_001999.3 transcript reference sequence.

543 entries on 6 pages. Showing entries 1 - 100.
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Effect     

Reported     

Exon     

AscendingDNA change (cDNA)     

RNA change     

Protein     

Classification method     

Clinical classification     

DNA change (genomic) (hg19)     

DNA change (hg38)     

Published as     

ISCN     

DB-ID     

Variant remarks     

Reference     

ClinVar ID     

dbSNP ID     

Origin     

Segregation     

Frequency     

Re-site     

VIP     

Methylation     

Owner     
./. 1 - c.-17843989_*3766623dup - - - pathogenic g.123828524_145717285dup - - - SIL1_000024 mosaicism, copy number 3 in 0.38 cells PubMed: DDDS 2015, Journal: DDDS 2015 - - Somatic - - - - - Johan den Dunnen
?/. 1 - c.5G>A r.(?) p.(Gly2Glu) - VUS g.127873292C>T - FBN2(NM_001999.3):c.5G>A (p.(Gly2Glu)) - FBN2_000496 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.26T>C r.(?) p.(Leu9Pro) - likely benign g.127873271A>G g.128537578A>G FBN2(NM_001999.4):c.26T>C (p.L9P) - FBN2_000221 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-?/., ?/. 2 1 c.68C>G r.(?) p.(Ala23Gly) - likely benign, VUS g.127873229G>C g.128537536G>C FBN2(NM_001999.3):c.68C>G (p.(Ala23Gly)) - FBN2_000220 VKGL data sharing initiative Nederland PubMed: Peeters 2021 - - CLASSIFICATION record, Germline - 1/913 controls - - - Johan den Dunnen, VKGL-NL_Leiden
?/. 2 - c.83G>A r.(?) p.(Gly28Asp) - VUS g.127873214C>T - FBN2(NM_001999.4):c.83G>A (p.(Gly28Asp), p.G28D) - FBN2_000495 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_VUmc
?/. 1 - c.87G>T r.(?) p.(Gln29His) - VUS g.127873210C>A - FBN2(NM_001999.3):c.87G>T (p.(Gln29His)) - FBN2_000494 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 1 c.92A>T r.(?) p.(Gln31Leu) - VUS g.127873205T>A g.128537512T>A - - FBN2_000329 - PubMed: Peeters 2021 - - Germline - 1/913 controls - - - Johan den Dunnen
-?/. 3 - c.110C>A r.(?) p.(Pro37Gln) - likely benign g.127873187G>T g.128537494G>T FBN2(NM_001999.3):c.110C>A (p.P37Q, p.(Pro37Gln)), FBN2(NM_001999.4):c.110C>A (p.P37Q) - FBN2_000144 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_VUmc
-/. 2 - c.111G>A r.(?) p.(Pro37=) - benign g.127873186C>T g.128537493C>T FBN2(NM_001999.4):c.111G>A (p.P37=) - FBN2_000143 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht, VKGL-NL_VUmc
?/. 1 - c.132G>C r.(?) p.(Pro44=) - VUS g.127873165C>G g.128537472C>G FBN2(NM_001999.3):c.132G>C (p.P44=) - FBN2_000142 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 2 - c.158G>A r.(?) p.(Gly53Asp) - VUS g.127873139C>T - FBN2(NM_001999.3):c.158G>A (p.G53D, p.(Gly53Asp)) - FBN2_000296 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam
?/. 1 - c.168C>T r.(?) p.(Gly56=) - VUS g.127873129G>A - FBN2(NM_001999.3):c.168C>T (p.G56=) - FBN2_000344 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.170G>T r.(?) p.(Gly57Val) - VUS g.127873127C>A - FBN2(NM_001999.4):c.170G>T (p.(Gly57Val)) - FBN2_000550 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-/. 2 - c.183C>G r.(?) p.(Pro61=) - benign g.127873114G>C g.128537421G>C FBN2(NM_001999.4):c.183C>G (p.P61=) - FBN2_000219 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht, VKGL-NL_VUmc
-?/. 1 - c.186G>A r.(?) p.(Glu62=) - likely benign g.127873111C>T g.128537418C>T FBN2(NM_001999.4):c.186G>A (p.E62=) - FBN2_000218 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. 1 - c.195G>A r.(?) p.(Glu65=) - likely benign g.127873102C>T g.128537409C>T FBN2(NM_001999.3):c.195G>A (p.E65=) - FBN2_000141 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/., -?/. 4 - c.203C>T r.(?) p.(Ala68Val) - benign, likely benign g.127873094G>A g.128537401G>A FBN2(NM_001999.3):c.203C>T (p.(Ala68Val)), FBN2(NM_001999.4):c.203C>T (p.A68V) - FBN2_000140 54 heterozygous, no homozygous; Clinindb (India), VKGL data sharing initiative Nederland PubMed: Narang 2020, Journal: Narang 2020 - rs62390671 CLASSIFICATION record, Germline - 54/2786 individuals - - - VKGL-NL_Leiden, VKGL-NL_Utrecht, VKGL-NL_VUmc, Mohammed Faruq
?/. 1 - c.230G>T r.(?) p.(Arg77Leu) - VUS g.127873067C>A - FBN2(NM_001999.4):c.230G>T (p.R77L) - FBN2_000312 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
?/. 2 - c.253G>C r.(?) p.(Gly85Arg) - VUS g.127873044C>G g.128537351C>G FBN2(NM_001999.3):c.253G>C (p.G85R, p.(Gly85Arg)) - FBN2_000217 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam
?/. 1 - c.254+2dup r.spl? p.? - VUS g.127873041dup - - - FBN2_000438 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-?/. 1 - c.254+5G>C r.spl? p.? - likely benign g.127873038C>G g.128537345C>G FBN2(NM_001999.3):c.254+5G>C (p.?) - FBN2_000216 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.254+43G>A r.(=) p.(=) - likely benign g.127873000C>T - FBN2(NM_001999.4):c.254+43G>A - FBN2_000437 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. 1 - c.254+373C>T r.(=) p.(=) - likely benign g.127872670G>A g.128536977G>A FBN2(NM_001999.4):c.254+373C>T - FBN2_000254 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
?/. 1 - c.287_289del r.(?) p.(Tyr96del) - VUS g.127872145_127872147del - FBN2(NM_001999.4):c.287_289delACT (p.Y96del) - FBN2_000311 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-?/. 1 - c.337+263T>C r.(=) p.(=) - likely benign g.127871832A>G - FBN2(NM_001999.4):c.337+263T>C - FBN2_000436 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
?/. 1 - c.376C>T r.(?) p.(Arg126Cys) - VUS g.127866348G>A g.128530655G>A FBN2(NM_001999.3):c.376C>T (p.(Arg126Cys)) - FBN2_000215 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.377G>A r.(?) p.(Arg126His) - likely benign g.127866347C>T - FBN2(NM_001999.4):c.377G>A (p.(Arg126His)) - FBN2_000467 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-/. 1 - c.436+16G>A r.(=) p.(=) - benign g.127866272C>T - FBN2(NM_001999.4):c.436+16G>A - FBN2_000493 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. 1 - c.437-4G>A r.spl? p.? - likely benign g.127863664C>T - FBN2(NM_001999.4):c.437-4G>A - FBN2_000539 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/., ?/. 4 4 c.518C>T r.(?) p.(Thr173Ile) - likely benign, VUS g.127863579G>A g.128527886G>A FBN2(NM_001999.3):c.518C>T (p.(Thr173Ile)), FBN2(NM_001999.4):c.518C>T (p.T173I) - FBN2_000214 VKGL data sharing initiative Nederland PubMed: Peeters 2021 - - CLASSIFICATION record, Germline - 1/714 controls - - - Johan den Dunnen, VKGL-NL_Leiden, VKGL-NL_Utrecht, VKGL-NL_VUmc
-?/. 1 - c.522T>C r.(?) p.(Tyr174=) - likely benign g.127863575A>G g.128527882A>G FBN2(NM_001999.3):c.522T>C (p.Y174=) - FBN2_000139 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 2 - c.579C>G r.(?) p.(Pro193=) - likely benign g.127855015G>C g.128519322G>C FBN2(NM_001999.3):c.579C>G (p.P193=), FBN2(NM_001999.4):c.579C>G (p.P193=) - FBN2_000213 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_VUmc
-?/. 1 - c.628+155T>A r.(=) p.(=) - likely benign g.127854811A>T g.128519118A>T FBN2(NM_001999.4):c.628+155T>A - FBN2_000253 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. 1 - c.629-18_629-15del r.(=) p.(=) - likely benign g.127800637_127800640del g.128464944_128464947del FBN2(NM_001999.4):c.629-18_629-15delTCTT - FBN2_000252 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-/. 1 - c.629-9A>G r.(=) p.(=) - benign g.127800623T>C g.128464930T>C FBN2(NM_001999.4):c.629-9A>G - FBN2_000212 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. 1 - c.629-6T>C r.(=) p.(=) - likely benign g.127800620A>G - FBN2(NM_001999.4):c.629-6T>C - FBN2_000466 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.671T>C r.(?) p.(Met224Thr) - likely benign g.127800572A>G - FBN2(NM_001999.3):c.671T>C (p.(Met224Thr)) - FBN2_000492 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.672G>T r.(?) p.(Met224Ile) - VUS g.127800571C>A - FBN2(NM_001999.4):c.672G>T (p.M224I) - FBN2_000511 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. 1 - c.696T>C r.(?) p.(Ile232=) - likely benign g.127800547A>G - FBN2(NM_001999.4):c.696T>C (p.I232=) - FBN2_000369 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/., ?/. 2 - c.704C>T r.(?) p.(Thr235Met) - likely benign, VUS g.127800539G>A - FBN2(NM_001999.3):c.704C>T (p.(Thr235Met)), FBN2(NM_001999.4):c.704C>T (p.T235M) - FBN2_000491 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_VUmc
-/., -?/., ?/. 7 6 c.728T>C r.(?) p.(Ile243Thr) - benign, likely benign, VUS g.127800515A>G g.128464822A>G FBN2(NM_001999.3):c.728T>C (p.(Ile243Thr)), FBN2(NM_001999.4):c.728T>C (p.I243T) - FBN2_000211 7 homozygous; Clinindb (India), 75 heterozygous; Clinindb (India), 1 more item PubMed: Narang 2020, Journal: Narang 2020, PubMed: Peeters 2021 - rs117524265 CLASSIFICATION record, Germline - 5/216 cases, 5/913 controls, 7/2795 individuals, 75/2795 individuals - - - Johan den Dunnen, VKGL-NL_Leiden, VKGL-NL_Utrecht, VKGL-NL_VUmc, Mohammed Faruq
-/., -?/. 4 - c.738G>A r.(?) p.(Ala246=) - benign, likely benign g.127800505C>T g.128464812C>T FBN2(NM_001999.3):c.738G>A (p.A246=, p.(Ala246=)), FBN2(NM_001999.4):c.738G>A (p.A246=) - FBN2_000138 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Utrecht, VKGL-NL_VUmc
?/. 2 - c.756G>T r.(?) p.(Glu252Asp) - VUS g.127800487C>A g.128464794C>A FBN2(NM_001999.3):c.756G>T (p.E252D), FBN2(NM_001999.4):c.756G>T (p.(Glu252Asp)) - FBN2_000137 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam
?/. 1 - c.763C>T r.(?) p.(Pro255Ser) - VUS g.127800480G>A - FBN2(NM_001999.4):c.763C>T (p.P255S) - FBN2_000510 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-?/., ?/. 2 - c.809G>A r.(?) p.(Arg270His) - likely benign, VUS g.127800434C>T g.128464741C>T FBN2(NM_001999.4):c.809G>A (p.(Arg270His), p.R270H) - FBN2_000210 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Groningen
?/. 1 - c.809G>T r.(?) p.(Arg270Leu) - VUS g.127800434C>A - FBN2(NM_001999.3):c.809G>T (p.(Arg270Leu)) - FBN2_000490 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 5 - c.811A>C r.(?) p.(Thr271Pro) - VUS g.127800432T>G g.128464739T>G FBN2(NM_001999.3):c.811A>C (p.T271P, p.(Thr271Pro)), FBN2(NM_001999.4):c.811A>C (p.T271P) - FBN2_000136 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Utrecht, VKGL-NL_Nijmegen, VKGL-NL_VUmc
?/. 1 - c.826+2T>C r.spl? p.? - VUS g.127800415A>G - FBN2(NM_001999.4):c.826+2T>C - FBN2_000393 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. 1 - c.826+15T>G r.(=) p.(=) - likely benign g.127800402A>C - FBN2(NM_001999.4):c.826+15T>G - FBN2_000368 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-?/. 1 - c.826+24A>G r.(=) p.(=) - likely benign g.127800393T>C - FBN2(NM_001999.4):c.826+24A>G - FBN2_000409 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. 1 - c.826+205G>A r.(=) p.(=) - likely benign g.127800212C>T - FBN2(NM_001999.4):c.826+205G>A - FBN2_000384 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. 1 - c.827-318A>T r.(=) p.(=) - likely benign g.127782617T>A - FBN2(NM_001999.4):c.827-318A>T - FBN2_000435 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. 1 - c.827-270G>A r.(=) p.(=) - likely benign g.127782569C>T - FBN2(NM_001999.4):c.827-270G>A - FBN2_000434 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. 1 - c.827-3C>T r.spl? p.? - likely benign g.127782302G>A g.128446609G>A FBN2(NM_001999.3):c.827-3C>T (p.?) - FBN2_000239 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/., ?/. 6 7 c.829G>A r.(?) p.(Val277Ile) - likely benign, VUS g.127782297C>T g.128446604C>T FBN2(NM_001999.3):c.829G>A (p.V277I, p.(Val277Ile)), FBN2(NM_001999.4):c.829G>A (p.V277I) - FBN2_000209 VKGL data sharing initiative Nederland PubMed: Peeters 2021 - - CLASSIFICATION record, Germline - 1/913 controls, 3/714 controls - - - Johan den Dunnen, VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Utrecht, VKGL-NL_VUmc
?/. 1 - c.884C>T r.(?) p.(Thr295Ile) - VUS g.127782242G>A g.128446549G>A FBN2(NM_001999.3):c.884C>T (p.T295I) - FBN2_000135 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.910C>T r.(?) p.(Pro304Ser) - VUS g.127782216G>A g.128446523G>A - - FBN2_000238 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-/. 1 - c.952+141A>C r.(=) p.(=) - benign g.127782033T>G g.128446340T>G FBN2(NM_001999.4):c.952+141A>C - FBN2_000134 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-/. 1 - c.952+4177C>T r.(=) p.(=) - benign g.127777997G>A g.128442304G>A FBN2(NM_001999.4):c.952+4177C>T - FBN2_000133 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-?/. 4 - c.953-8T>G r.(=) p.(=) - likely benign g.127744500A>C g.128408807A>C FBN2(NM_001999.3):c.953-8T>G (p.(=)), FBN2(NM_001999.4):c.953-8T>G - FBN2_000132 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Utrecht, VKGL-NL_VUmc
-?/. 1 - c.957T>C r.(?) p.(Ile319=) - likely benign g.127744488A>G g.128408795A>G FBN2(NM_001999.4):c.957T>C (p.I319=) - FBN2_000131 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/., ?/. 2 8 c.968G>A r.(?) p.(Ser323Asn) - likely benign, VUS g.127744477C>T g.128408784C>T FBN2(NM_001999.4):c.968G>A (p.S323N) - FBN2_000295 VKGL data sharing initiative Nederland PubMed: Peeters 2021 - - CLASSIFICATION record, Germline - 1/913 controls - - - Johan den Dunnen, VKGL-NL_Utrecht
-/., -?/., ?/. 8 8 c.976C>T r.(?) p.(Pro326Ser) - benign, likely benign, VUS g.127744469G>A g.128408776G>A 976G>A, 1 more item - FBN2_000130 VKGL data sharing initiative Nederland PubMed: Duvvari 2016, PubMed: Peeters 2021 - rs28763954 CLASSIFICATION record, Germline - 1/216 cases, 1/714 controls, 2/913 controls - - - Johan den Dunnen, VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Utrecht, VKGL-NL_VUmc
-?/. 3 - c.1011C>T r.(?) p.(Thr337=) - likely benign g.127744434G>A g.128408741G>A FBN2(NM_001999.3):c.1011C>T (p.T337=), FBN2(NM_001999.4):c.1011C>T (p.T337=) - FBN2_000129 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Nijmegen, VKGL-NL_VUmc
-?/., ?/. 9 8 c.1040G>A r.(?) p.(Arg347His) - likely benign, VUS g.127744405C>T g.128408712C>T FBN2(NM_001999.3):c.1040G>A (p.R347H, p.(Arg347His)), FBN2(NM_001999.4):c.1040G>A (p.R347H) - FBN2_000208 13 heterozygous, no homozygous; Clinindb (India), VKGL data sharing initiative Nederland PubMed: Narang 2020, Journal: Narang 2020, PubMed: Peeters 2021, PubMed: Schubert 2016 - rs112428886 CLASSIFICATION record, Germline - 13/2795 individuals, 3/913 controls, 4/216 cases, 5/714 controls - - - Johan den Dunnen, VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Utrecht, VKGL-NL_VUmc, Mohammed Faruq
+/. 1 - c.1064G>A r.(?) p.(Gly355Asp) - pathogenic (recessive) g.127744381C>T g.128408688C>T - - FBN2_000147 - PubMed: Alazami 2015, Journal: Alazami 2015 - - Germline yes - - - - Johan den Dunnen
-?/. 1 - c.1078+52A>G r.(=) p.(=) - likely benign g.127744315T>C - FBN2(NM_001999.4):c.1078+52A>G - FBN2_000383 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. 1 - c.1078+266A>T r.(=) p.(=) - likely benign g.127744101T>A - FBN2(NM_001999.4):c.1078+266A>T - FBN2_000538 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.1079-217T>C r.(=) p.(=) - likely benign g.127731184A>G - FBN2(NM_001999.4):c.1079-217T>C - FBN2_000465 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. 1 - c.1079-211C>A r.(=) p.(=) - likely benign g.127731178G>T - FBN2(NM_001999.4):c.1079-211C>A - FBN2_000464 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. 1 - c.1079-14T>G r.(=) p.(=) - likely benign g.127730981A>C g.128395288A>C - - FBN2_000237 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 - c.1120C>T r.(?) p.(Arg374Cys) - VUS g.127730926G>A - FBN2(NM_001999.4):c.1120C>T (p.(Arg374Cys)) - FBN2_000537 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.1121G>A r.(?) p.(Arg374His) - VUS g.127730925C>T g.128395232C>T FBN2(NM_001999.3):c.1121G>A (p.(Arg374His)) - FBN2_000251 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 2 9 c.1139C>T r.(?) p.(Pro380Leu) - VUS g.127730907G>A g.128395214G>A FBN2(NM_001999.4):c.1139C>T (p.P380L) - FBN2_000328 VKGL data sharing initiative Nederland PubMed: Peeters 2021 - - CLASSIFICATION record, Germline - 1/913 controls - - - Johan den Dunnen, VKGL-NL_Groningen
-?/. 1 - c.1148T>A r.(?) p.(Met383Lys) - likely benign g.127730898A>T g.128395205A>T FBN2(NM_001999.4):c.1148T>A (p.M383K) - FBN2_000207 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
?/. 1 9 c.1157T>C r.(?) p.(Met386Thr) - VUS g.127730889A>G g.128395196A>G - - FBN2_000327 - PubMed: Peeters 2021 - - Germline - 1/913 controls - - - Johan den Dunnen
?/. 1 - c.1180C>T r.(?) p.(Arg394Cys) - VUS g.127730866G>A - FBN2(NM_001999.3):c.1180C>T (p.(Arg394Cys)) - FBN2_000489 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.1194C>T r.(?) p.(=) - likely benign g.127730852G>A - FBN2(NM_001999.4):c.1194C>T (p.I398=) - FBN2_000522 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
?/. 1 - c.1216C>T r.(?) p.(Pro406Ser) - VUS g.127730830G>A - FBN2(NM_001999.3):c.1216C>T (p.(Pro406Ser)) - FBN2_000488 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. 1 - c.1234G>A r.(?) p.(Glu412Lys) - VUS g.127729059C>T g.128393366C>T - - FBN2_000501 - PubMed: Mansoorshahi 2024 - rs962522833 Germline - - - - - Johan den Dunnen
?/. 2 10 c.1238A>G r.(?) p.(Tyr413Cys) - VUS g.127729055T>C g.128393362T>C FBN2(NM_001999.4):c.1238A>G (p.(Tyr413Cys)) - FBN2_000326 VKGL data sharing initiative Nederland PubMed: Peeters 2021 - - CLASSIFICATION record, Germline - 2/714 controls - - - Johan den Dunnen, VKGL-NL_Leiden
-?/., ?/. 2 10 c.1285A>G r.(?) p.(Ser429Gly) - likely benign, VUS g.127729008T>C g.128393315T>C FBN2(NM_001999.4):c.1285A>G (p.S429G) - FBN2_000325 VKGL data sharing initiative Nederland PubMed: Peeters 2021 - - CLASSIFICATION record, Germline - 1/714 controls - - - Johan den Dunnen, VKGL-NL_VUmc
-?/. 1 - c.1411G>A r.(?) p.(Val471Ile) - likely benign g.127728882C>T - - - FBN2_000362 - - - rs138046782 Unknown - - - - - MobiDetails
-?/. 1 - c.1422C>T r.(?) p.(Ala474=) - likely benign g.127728871G>A g.128393178G>A FBN2(NM_001999.3):c.1422C>T (p.A474=) - FBN2_000250 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 2 - c.1423G>C r.(?) p.(Gly475Arg) - VUS g.127728870C>G g.128393177C>G FBN2(NM_001999.4):c.1423G>C (p.G475R) - FBN2_000206 VKGL data sharing initiative Nederland PubMed: Overwater 2018 - - CLASSIFICATION record, Germline - 1/128 cases FA - - - Johan den Dunnen, VKGL-NL_VUmc
-?/. 1 - c.1431G>A r.(?) p.(=) - likely benign g.127728862C>T - FBN2(NM_001999.4):c.1431G>A (p.G477=) - FBN2_000433 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
?/. 2 10 c.1435G>A r.(?) p.(Gly479Arg) - VUS g.127728858C>T g.128393165C>T FBN2(NM_001999.3):c.1435G>A (p.G479R) - FBN2_000128 VKGL data sharing initiative Nederland PubMed: Peeters 2021 - - CLASSIFICATION record, Germline - 1/913 controls - - - Johan den Dunnen, VKGL-NL_Rotterdam
?/. 1 - c.1453A>G r.(?) p.(Ile485Val) - VUS g.127728840T>C g.128393147T>C FBN2(NM_001999.3):c.1453A>G (p.I485V) - FBN2_000127 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.1465+94T>C r.(=) p.(=) - likely benign g.127728734A>G - FBN2(NM_001999.4):c.1465+94T>C - FBN2_000432 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. 1 - c.1465+177A>G r.(=) p.(=) - likely benign g.127728651T>C - FBN2(NM_001999.4):c.1465+177A>G - FBN2_000294 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. 1 - c.1465+247A>C r.(=) p.(=) - likely benign g.127728581T>G - FBN2(NM_001999.4):c.1465+247A>C - FBN2_000431 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-/., -?/. 2 - c.1466-5C>T r.spl? p.? - benign, likely benign g.127727853G>A g.128392160G>A FBN2(NM_001999.3):c.1466-5C>T (p.?), FBN2(NM_001999.4):c.1466-5C>T - FBN2_000126 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_VUmc
-?/. 4 - c.1466-4G>A r.spl? p.? - likely benign g.127727852C>T g.128392159C>T FBN2(NM_001999.3):c.1466-4G>A (p.?), FBN2(NM_001999.4):c.1466-4G>A - FBN2_000205 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Utrecht, VKGL-NL_VUmc
-?/. 1 - c.1482A>T r.(?) p.(Thr494=) - likely benign g.127727832T>A g.128392139T>A FBN2(NM_001999.3):c.1482A>T (p.T494=) - FBN2_000204 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 2 - c.1484T>C r.(?) p.(Ile495Thr) - VUS g.127727830A>G g.128392137A>G FBN2(NM_001999.4):c.1484T>C (p.I495T) - FBN2_000145 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen, VKGL-NL_VUmc
?/. 1 - c.1499A>C r.(?) p.(His500Pro) - VUS g.127727815T>G g.128392122T>G FBN2(NM_001999.3):c.1499A>C (p.H500P) - FBN2_000203 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 2 - c.1500T>G r.(?) p.(His500Gln) - likely benign g.127727814A>C - FBN2(NM_001999.4):c.1500T>G (p.H500Q) - FBN2_000293 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht, VKGL-NL_Nijmegen
+?/. 1 - c.1526G>T r.(?) p.(Arg509Leu) ACMG likely pathogenic (dominant) g.127727788C>A g.128392095C>A - - FBN2_000346 ACMG PM2, PM6, PP3, PP4 PubMed: Marinakis 2021 - rs199587570 Germline/De novo (untested) - - - - - Jan Traeger-Synodinos
-?/. 1 - c.1543T>A r.(?) p.(Ser515Thr) - likely benign g.127727771A>T - FBN2(NM_001999.4):c.1543T>A (p.(Ser515Thr)) - FBN2_000463 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.1553G>A r.(?) p.(Arg518Gln) - likely benign g.127727761C>T - FBN2(NM_001999.3):c.1553G>A (p.(Arg518Gln)) - FBN2_000487 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
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