All variants in the FBN1 gene

FBN1 variants classified by the Clinical Genome Resource's FBN1 variant curation expert panel.
Information The variants shown are described using the NM_000138.4 transcript reference sequence.

60 entries on 1 page. Showing entries 1 - 60.
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Effect     

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     
+/. - c.1A>C r.(1A>C) p.(Met1?) ACMG pathogenic g.48936966T>G g.48644769T>G - - FBN1_001596 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-495569  - SUMMARY record - - - - - Johan den Dunnen
?/. - c.59A>G r.(59A>G) p.(Tyr20Cys) ACMG VUS g.48936908T>C g.48644711T>C - - FBN1_000202 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-161245 - SUMMARY record - - - - - Johan den Dunnen
-/. - c.79G>A r.(79G>A) p.(Ala27Thr) ACMG benign g.48936888C>T g.48644691C>T - - FBN1_000201 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-163486 - SUMMARY record - - - - - Johan den Dunnen
+?/. - c.629G>A r.(629G>A) p.(Cys210Tyr) ACMG likely pathogenic g.48829915C>T g.48537718C>T - - FBN1_000812 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-426140 - SUMMARY record - - - - - Johan den Dunnen
+?/. - c.799_805del r.(799_805del) p.(Gly267LeufsTer61) ACMG likely pathogenic g.48826336_48826342del g.48534139_48534145del - - FBN1_001595 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-406374 - SUMMARY record - - - - - Johan den Dunnen
-?/. - c.985A>G r.(985A>G) p.(Ile329Val) ACMG likely benign g.48818330T>C g.48526133T>C - - FBN1_001594 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-549475 - SUMMARY record - - - - - Johan den Dunnen
-/. - c.986T>C r.(986T>C) p.(Ile329Thr) ACMG benign g.48818329A>G g.48526132A>G - - FBN1_000186 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-36133 - SUMMARY record - - - - - Johan den Dunnen
?/. - c.1961-3T>G r.spl? p.? ACMG VUS g.48796139A>C g.48503942A>C - - FBN1_001593 not yet formally curated PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-16445 - SUMMARY record - - - - - Johan den Dunnen
+?/. - c.2034_2052del r.(2034_2052del) p.(Thr679AlafsTer33) ACMG likely pathogenic g.48796051_48796069del g.48503854_48503872del - - FBN1_001592 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-2413171 - SUMMARY record - - - - - Johan den Dunnen
+?/. - c.2364dup r.(2364dup) p.(Val789CysfsTer13) ACMG likely pathogenic g.48788355dup g.48496158dup - - FBN1_001591 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-2413175 - SUMMARY record - - - - - Johan den Dunnen
+?/. - c.2669G>A r.(2669G>A) p.(Cys890Tyr) ACMG likely pathogenic g.48787328C>T g.48495131C>T - - FBN1_000686 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-431935 - SUMMARY record - - - - - Johan den Dunnen
?/. - c.2678-15C>A r.spl? p.? ACMG VUS g.48786466G>T g.48494269G>T - - FBN1_001590 not yet formally curated PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-446494 - SUMMARY record - - - - - Johan den Dunnen
-/. - c.2956G>A r.(2956G>A) p.(Ala986Thr) ACMG benign g.48782174C>T g.48489977C>T - - FBN1_000663 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-36060 - SUMMARY record - - - - - Johan den Dunnen
-?/. - c.3058A>G r.(3058A>G) p.(Thr1020Ala) ACMG likely benign g.48782072T>C g.48489875T>C - - FBN1_000659 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-42325 - SUMMARY record - - - - - Johan den Dunnen
?/. - c.3295G>A r.(3295G>A) p.(Glu1099Lys) ACMG VUS g.48780352C>T g.48488155C>T - - FBN1_001085 not yet formally curated PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-457191 - SUMMARY record - - - - - Johan den Dunnen
-/. - c.3422C>T r.(3422C>T) p.(Pro1141Leu) ACMG benign g.48779550G>A g.48487353G>A - - FBN1_000154 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-42334 - SUMMARY record - - - - - Johan den Dunnen
-/. - c.3509G>A r.(3509G>A) p.(Arg1170His) ACMG benign g.48779352C>T g.48487155C>T - - FBN1_000147 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-16451 - SUMMARY record - - - - - Johan den Dunnen
+/. - c.3603C>A r.(3603C>A) p.(Cys1201Ter) ACMG pathogenic g.48777680G>T g.48485483G>T - - FBN1_001589 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-2581043 - SUMMARY record - - - - - Johan den Dunnen
+?/. - c.3623del r.(3623del) p.(Cys1208LeufsTer22) ACMG likely pathogenic g.48777660del g.48485463del - - FBN1_001588 not yet formally curated PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-549177 - SUMMARY record - - - - - Johan den Dunnen
+?/. - c.3632_3634del r.(3632_3634del) p.(Phe1211del) ACMG likely pathogenic g.48777651_48777653del g.48485454_48485456del - - FBN1_001587 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-549178 - SUMMARY record - - - - - Johan den Dunnen
-?/. - c.3689T>C r.(3689T>C) p.(Met1230Thr) ACMG likely benign g.48777594A>G g.48485397A>G - - FBN1_000598 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-263789 - SUMMARY record - - - - - Johan den Dunnen
+/. - c.4149_4154del r.(4149_4154del) p.(Met1384_Gly1385del) ACMG pathogenic g.48766508_48766513del g.48474311_48474316del - - FBN1_001586 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-2413170 - SUMMARY record - - - - - Johan den Dunnen
?/. - c.4214T>G r.(4214T>G) p.(Leu1405Arg) ACMG VUS g.48764870A>C g.48472673A>C - - FBN1_000558 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-200041 - SUMMARY record - - - - - Johan den Dunnen
+/. - c.4255C>T r.(4255C>T) p.(Gln1419Ter) ACMG pathogenic g.48764829G>A g.48472632G>A - - FBN1_001585 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-2413173 - SUMMARY record - - - - - Johan den Dunnen
-?/. - c.4270C>G r.(4270C>G) p.(Pro1424Ala) ACMG likely benign g.48764814G>C g.48472617G>C - - FBN1_000552 not yet formally curated PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-42355 - SUMMARY record - - - - - Johan den Dunnen
+/. - c.4414T>C r.(4414T>C) p.(Cys1472Arg) ACMG pathogenic g.48762876A>G g.48470679A>G - - FBN1_001584 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-549229 - SUMMARY record - - - - - Johan den Dunnen
+/. - c.4459G>A r.(4459G>A) p.(Asp1487Asn) ACMG pathogenic g.48762831C>T g.48470634C>T - - FBN1_001583 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-549232 - SUMMARY record - - - - - Johan den Dunnen
?/. - c.4466A>G r.(4466A>G) p.(Asn1489Ser) ACMG VUS g.48760725T>C g.48468528T>C - - FBN1_000529 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-373598 - SUMMARY record - - - - - Johan den Dunnen
+?/. - c.4583-5A>G r.spl p.? ACMG likely pathogenic g.48760304T>C g.48468107T>C - - FBN1_001582 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-406332 - SUMMARY record - - - - - Johan den Dunnen
-/. - c.4640C>T r.(4640C>T) p.(Thr1547Ile) ACMG benign g.48760242G>A g.48468045G>A - - FBN1_000515 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-42367 - SUMMARY record - - - - - Johan den Dunnen
+?/. - c.4700del r.(4700del) p.(Gly1567ValfsTer14) ACMG likely pathogenic g.48760184del g.48467987del - - FBN1_001581 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-626879 - SUMMARY record - - - - - Johan den Dunnen
?/. - c.4935G>A r.spl? p.? ACMG VUS g.48757772C>T g.48465575C>T - - FBN1_001580 not yet formally curated PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-263692 - SUMMARY record - - - - - Johan den Dunnen
?/. - c.5443G>A r.(5443G>A) p.(Gly1815Ser) ACMG VUS g.48744861C>T g.48452664C>T - - FBN1_000474 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-200064 - SUMMARY record - - - - - Johan den Dunnen
+?/. - c.5734_5736del r.(5734_5736del) p.(Phe1912del) ACMG likely pathogenic g.48738956_48738958del g.48446759_48446761del - - FBN1_001578 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-2506004 - SUMMARY record - - - - - Johan den Dunnen
+/. - c.5743C>T r.(5743C>T) p.(Arg1915Cys) ACMG pathogenic g.48738948G>A g.48446751G>A - - FBN1_001577 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-495629 - SUMMARY record - - - - - Johan den Dunnen
+/. - c.5788+5G>A r.spl p.? ACMG pathogenic g.48738898C>T g.48446701C>T - - FBN1_001101 not yet formally curated PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-42394 - SUMMARY record - - - - - Johan den Dunnen
-?/. - c.6073G>T r.(6073G>T) p.(Ala2025Ser) ACMG likely benign g.48734008C>A g.48441811C>A - - FBN1_000123 not yet formally curated PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-36097 - SUMMARY record - - - - - Johan den Dunnen
+/. - c.6453C>T r.spl p.? ACMG pathogenic g.48729201G>A g.48437004G>A - - FBN1_000118 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-200193 - SUMMARY record - - - - - Johan den Dunnen
+?/. - c.6505G>T r.(6505G>T) p.(Glu2169Ter) ACMG likely pathogenic g.48726902C>A g.48434705C>A - - FBN1_001576 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-2413172 - SUMMARY record - - - - - Johan den Dunnen
+?/. - c.7003C>T r.(7003C>T) p.(Arg2335Trp) ACMG likely pathogenic g.48719965G>A g.48427768G>A - - FBN1_001575 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-200100 - SUMMARY record - - - - - Johan den Dunnen
+/. - c.7039_7040del r.(7039_7040del) p.(Met2347ValfsTer19) ACMG pathogenic g.48719928_48719929del g.48427731_48427732del - - FBN1_001227 not yet formally curated PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-200171 - SUMMARY record - - - - - Johan den Dunnen
+/. - c.7167_7168del r.(7167_7168del) p.(Cys2390SerfsTer15) ACMG pathogenic g.48719802_48719803del g.48427605_48427606del - - FBN1_001574 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-42420 - SUMMARY record - - - - - Johan den Dunnen
+/. - c.7204+1G>T r.spl p.? ACMG pathogenic g.48719763C>A g.48427566C>A - - FBN1_001573 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-549394 - SUMMARY record - - - - - Johan den Dunnen
+?/. - c.7259A>G r.(7259A>G) p.(Asn2420Ser) ACMG likely pathogenic g.48718007T>C g.48425810T>C - - FBN1_001572 not yet formally curated PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-1319506 - SUMMARY record - - - - - Johan den Dunnen
+/. - c.7361_7364delinsA r.(7361_7364delinsA) p.(Pro2454_Cys2455delinsHis) ACMG pathogenic g.48717655_48717658delinsT g.48425458_48425461delinsT - - FBN1_001571 not yet formally curated PubMed: Drackley 2024, Journal: Drackley 2024 - - SUMMARY record - - - - - Johan den Dunnen
+/. - c.7383del r.(7383del) p.(Asn2461LysfsTer221) ACMG pathogenic g.48717636del g.48425439del - - FBN1_001570 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-1325453 - SUMMARY record - - - - - Johan den Dunnen
?/. - c.7412C>G r.(7412C>G) p.(Pro2471Arg) ACMG VUS g.48717607G>C g.48425410G>C - - FBN1_000099 not yet formally curated PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-36115 - SUMMARY record - - - - - Johan den Dunnen
+/. - c.7540G>A r.(7540G>A) p.(Gly2514Arg) ACMG pathogenic g.48714179C>T g.48421982C>T - - FBN1_000299 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-178034 - SUMMARY record - - - - - Johan den Dunnen
+/. - c.7540G>T r.(7540G>T) p.(Gly2514Ter) ACMG pathogenic g.48714179C>A g.48421982C>A - - FBN1_001569 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-2506018 - SUMMARY record - - - - - Johan den Dunnen
+?/. - c.7577A>G r.(7577A>G) p.(Asn2526Ser) ACMG likely pathogenic g.48713877T>C g.48421680T>C - - FBN1_000298 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-200198 - SUMMARY record - - - - - Johan den Dunnen
?/. - c.7585A>C r.(7585A>C) p.(Thr2529Pro) ACMG VUS g.48713869T>G g.48421672T>G - - FBN1_001568 not yet formally curated PubMed: Drackley 2024, Journal: Drackley 2024 - - SUMMARY record - - - - - Johan den Dunnen
?/. - c.7643T>A r.(7643T>A) p.(Phe2548Tyr) ACMG VUS g.48713811A>T g.48421614A>T - - FBN1_001567 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-2413174 - SUMMARY record - - - - - Johan den Dunnen
+?/. - c.7692_7693delinsGGTGATCTGGAAGGTGAA r.(7692_7693delinsGGTGATCTGGAAGGTGAA) p.(Ser2564ArgfsTer8) ACMG likely pathogenic g.48713761_48713762delinsTTCACCTTCCAGATCACC g.48421564_48421565delinsTTCACCTTCCAGATCACC - - FBN1_001566 not yet formally curated PubMed: Drackley 2024, Journal: Drackley 2024 - - SUMMARY record - - - - - Johan den Dunnen
+/. - c.8051delinsTT r.(8051delinsTT) p.(Gly2684ValfsTer21) ACMG pathogenic g.48707733delinsAA g.48415536delinsAA - - FBN1_001565 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-549451 - SUMMARY record - - - - - Johan den Dunnen
+/. - c.8080C>T r.(8080C>T) p.(Arg2694Ter) ACMG pathogenic g.48704912G>A g.48412715G>A - - FBN1_001564 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-163461 - SUMMARY record - - - - - Johan den Dunnen
-?/. - c.8149G>A r.(8149G>A) p.(Glu2717Lys) ACMG likely benign g.48704843C>T g.48412646C>T - - FBN1_000248 not yet formally curated PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-237106 - SUMMARY record - - - - - Johan den Dunnen
+?/. - c.8176C>T r.(8176C>T) p.(Arg2726Trp) ACMG benign g.48704816G>A g.48412619G>A - - FBN1_000246 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-16445 - SUMMARY record - - - - - Johan den Dunnen
+/. - c.8326C>T r.(8326C>T) p.(Arg2776Ter) ACMG pathogenic g.48703477G>A g.48411280G>A - - FBN1_001225 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-16439 - SUMMARY record - - - - - Johan den Dunnen
-/. - c.8363C>T r.(8363C>T) p.(Thr2788Met) ACMG benign g.48703440G>A g.48411243G>A - - FBN1_000234 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-263832 - SUMMARY record - - - - - Johan den Dunnen
+/. - c.8525_8529del r.(8525_8529del) p.(Leu2842ProfsTer7) ACMG pathogenic g.48703277_48703281del g.48411080_48411084del - - FBN1_001563 - PubMed: Drackley 2024, Journal: Drackley 2024 ClinVar-419823 - SUMMARY record - - - - - Johan den Dunnen
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