Unique variants in the BBS5 gene

This database is one of the "Eye disease" gene variant databases.
Information The variants shown are described using the NM_152384.2 transcript reference sequence.

88 entries on 1 page. Showing entries 1 - 88.
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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.-40= r.(?) p.(=) - benign g.170336024= g.169479514= NM_152384:c.1-40C>G - BBS5_000072 - PubMed: Smaoui 2006 - rs1879466 Germline - 3/19 families BBS - - - Johan den Dunnen
-/. 1 - c.-40G>C r.(?) p.(=) - benign g.170336024G>C g.169479514G>C - - BBS5_000019 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 _1_12_ c.-118_*2073{2} r.? p.? - VUS g.(?_170336059)_(170361097_?)dup - chr2:170336059–170361097 - BBS5_000065 - PubMed: Ellingsford 2018 - - Germline - - - - - LOVD
+/., +?/., ?/. 8 - c.? r.(?), r.? p.? ACMG likely pathogenic, pathogenic, VUS g.170343574_170343578insN[(400_500)], g.? g.? BBS5 263-271 indelGCTCTTA, BBS5:p.[N184S];[=], c.619–1G>C, [p.A242S], [p.I389I], [p.N184S], 1 more item - SNRNP200_000007 ACMG PM2, normal 2nd chromosome, 1 more item PubMed: Chen-2011, PubMed: Imhoff-2011, PubMed: Li 2004, PubMed: Redin-2012, PubMed: Weisschuh 2024 RCV000006534.4 - Germline, Unknown yes - - - - Johan den Dunnen
+/. 1 1 c.1A>T r.? p.? - pathogenic g.170336064A>T - c.1A>T - BBS5_000055 - PubMed: Muller-2010 - - Germline - - - - - LOVD
?/. 1 1 c.1A>Y r.? p.? - VUS g.170336064A>T - p.M1L - BBS5_000055 - PubMed: Scheidecker 2015 - - Germline - - - - - LOVD
+/., +?/. 5 1 c.2T>A r.(?), r.? p.(Met1?), p.? - likely pathogenic, pathogenic g.170336065T>A g.169479555T>A 2T/A (M1K), BBS5;NM_152384.2, c.[2T>A];[2T>A];p.[(Met1?)];[(Met1?)]; - BBS5_000047 homozygous PubMed: Ellingford 2016, PubMed: Harville-2010, PubMed: Jiman 2020 - - Germline, Unknown ?, yes 0/90 ethnically matched controls - - - LOVD
+/. 1 - c.2T>C r.(?) p.(Met1?) - pathogenic g.170336065T>C g.169479555T>C - - BBS5_000020 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 2 - c.25G>A r.(?) p.(Glu9Lys) - VUS g.170336088G>A g.169479578G>A BBS5(NM_152384.3):c.25G>A (p.(Glu9Lys)) - BBS5_000021 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Nijmegen
+?/. 1 1 c.54dup r.(?) p.(Ala19Argfs*14) - likely pathogenic g.170336117dup - c.54dupC - BBS5_000058 - PubMed: Feuillan-2011 - - Germline - - - - - LOVD
-/. 1 - c.60-176dup r.(=) p.(=) - benign g.170338585dup g.169482075dup BBS5(NM_152384.3):c.60-176dupA - BBS5_000030 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-?/. 2 - c.60-3T>C r.spl? p.? - likely benign g.170338758T>C g.169482248T>C BBS5(NM_152384.2):c.60-3T>C, BBS5(NM_152384.3):c.60-3T>C - BBS5_000031 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_AMC
?/. 2 - c.92T>C r.(?) p.(Ile31Thr) - VUS g.170338793T>C g.169482283T>C BBS5(NM_152384.3):c.92T>C (p.(Ile31Thr)) - BBS5_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Nijmegen
-/. 1 - c.108C>T r.(?) p.(Ser36=) - benign g.170338809C>T g.169482299C>T BBS5(NM_152384.2):c.108C>T (p.S36=) - BBS5_000032 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.110T>C r.(?) p.(Ile37Thr) - VUS g.170338811T>C - BBS5(NM_152384.3):c.110T>C (p.(Ile37Thr)) - BBS5_000082 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/., +?/. 7 2 c.123del r.(?) p.(Gly42Glufs*11), p.(Gly42GlufsTer11) - likely pathogenic, pathogenic, pathogenic (recessive) g.170338824del g.169482314del 123delA, BBS5 c.[123delA];[123delA], p.[G42Efs*11];[ G42Efs*11], c.123delA, c.[123del];[123del], 1 more item - BBS5_000006 homozygous, VKGL data sharing initiative Nederland PubMed: Habibi 2020, PubMed: Mary-2019, PubMed: Muller-2010, PubMed: Smaoui 2006 - - CLASSIFICATION record, Germline ? - - - - Johan den Dunnen, VKGL-NL_Utrecht, VKGL-NL_AMC
?/. 1 2 c.132T>G r.(?) p.(Asn44Lys) - VUS g.170338833T>G - c.132T>G - BBS5_000059 - PubMed: Abu-Safieh-2012, Abu-Safieh 2010 - - Germline - - - - - LOVD
?/. 1 - c.142G>A r.(?) p.(Gly48Ser) - VUS g.170338843G>A - BBS5(NM_152384.2):c.142G>A (p.G48S) - BBS5_000044 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. 1 - c.142+160C>A r.(?) p.(=) - benign g.170339003C>A g.169482493C>A - - BBS5_000073 - PubMed: Smaoui 2006 - rs2353187 Germline - 2/19 families BBS - - - Johan den Dunnen
+?/. 2 - c.143-4_143-3ins440bp r.spl? p.(?) - likely pathogenic g.? g.? BBS5, variant 1: c.143-4_143-3ins440bp/p.?, variant 2: c.143-4_143-3ins440bp/p.? - SNRNP200_000007 inserted nucleotides not given in the publication, possibly solved, homozygous PubMed: Weisschuh 2020 - - Germline yes - - - - LOVD
+/. 2 - c.143-1G>A r.(?) p.? - pathogenic g.170343578G>A g.169487068G>A BBS5 c.143-1 G > A (splice acceptor) - BBS5_000078 heterozygous PubMed: Torrefranca 2020 - - Germline yes - - - - LOVD
+/., +?/. 2 - c.143-1G>C r.spl p.(?), p.? ACMG likely pathogenic, pathogenic (recessive) g.170343578G>C g.169487068G>C BBS5, c.143-1G>C, p.?, homozygous - BBS5_000066 - Villafuerte-de la Cruz RA, et al., 2023. Submitted, PubMed: Perea-Romero 2021 ClinVar-569727 rs1054138918 Germline yes - - - - Rocio Villafuerte-de la Cruz
+/., +?/? 2 3 c.148C>A r.(?) p.(Leu50Ile) - pathogenic, VUS g.170343584C>A g.169487074C>A c.148C>A - BBS5_000001 - PubMed: Wang-2014 - - Unknown - - - - - Feng Wang
+/. 4 3 c.149T>G r.(?) p.(Leu50Arg) - pathogenic, pathogenic (recessive) g.170343585T>G - c.149T >G, c.149T>G, c.[149T>G];[149T>G] - BBS5_000051 - PubMed: Chen-2011, PubMed: M'hamdi 2014, PubMed: M'hamdi-2014, PubMed: Redin-2012 - - Germline, Unknown yes - - - - LOVD
+?/. 1 3 c.158C>T r.(?) p.(Thr53Ile) - likely pathogenic g.170343594C>T - c.158C>T p.(T53I) - BBS5_000060 - PubMed: Abu-Safieh-2012 - - Germline - 0/96 ethnically matched controls - - - LOVD
+/. 3 3 c.166A>G r.(?) p.(Arg56Gly) - pathogenic, pathogenic (recessive) g.170343602A>G - c.166A>G, c.[166A>G];[166A>G] - BBS5_000056 - PubMed: Mary-2019, PubMed: Muller-2010 - - Germline - - - - - LOVD
+?/. 1 - c.177G>A r.(?) p.(Trp59Ter) - likely pathogenic g.170343613G>A g.169487103G>A BBS5 W59X - BBS5_000067 homozygous PubMed: Li 2004 - - Germline yes - - - - LOVD
?/. 1 - c.178C>T r.(?) p.(His60Tyr) - VUS g.170343614C>T g.169487104C>T BBS5(NM_152384.2):c.178C>T (p.H60Y) - BBS5_000033 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.185T>C r.(?) p.(Leu62Ser) - VUS g.170343621T>C - BBS5(NM_152384.2):c.185T>C (p.L62S) - BBS5_000045 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 2 - c.196del r.(?) p.(Arg66Glufs*12) - likely pathogenic g.170343632del g.169487122del c.196delA;, p.(Arg66Glufs*12) - BBS5_000057 - PubMed: Khan 2019 - - Germline/De novo (untested) ? - - - - LOVD
?/. 1 - c.203A>G r.(?) p.(Asn68Ser) - VUS g.170343639A>G - BBS5(NM_152384.3):c.203A>G (p.(Asn68Ser)) - BBS5_000083 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. 2 12 c.208+2T>C r.(?) p.? - likely pathogenic g.170343646T>C g.169487136T>C BBS5 c.208+2T>C - BBS5_000068 homozygous PubMed: Imani 2019 - - Germline yes - - - - LOVD
+/., +?/. 8 - c.209-2A>G r.spl, r.spl? p.? - likely pathogenic, pathogenic g.170344314A>G g.169487804A>G BBS5(NM_152384.2):c.209-2A>G, BBS5(NM_152384.3):c.209-2A>G - BBS5_000007 VKGL data sharing initiative Nederland PubMed: Haer-Wigman 2017 - - CLASSIFICATION record, Germline yes - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Utrecht, VKGL-NL_Nijmegen
+/., +?/. 9 4 c.214G>A r.(?) p.(Gly72Ser) ACMG likely pathogenic, pathogenic g.170344321G>A g.169487811G>A BBS5 c.214G>A, p.(Gly72Ser), c.214G>A, p.(Gly72Ser), RP1 c.6171_6174del, p.(Thr2058Metfs*14), 5 more items - BBS5_000008 homozygous, VKGL data sharing initiative Nederland PubMed: Deveault-2011, PubMed: Habibi 2020, PubMed: Hjortshoj 2008, PubMed: Janssen-2011, 1 more item - - CLASSIFICATION record, Germline, Unknown ?, yes 0.019, 0/43 East Africans (Kenya) and 0/58 West Africans (Nigeria) - - - VKGL-NL_VUmc
+/. 1 - c.219C>G r.(?) p.(Tyr73*) - pathogenic g.170344326C>G - BBS5(NM_152384.3):c.219C>G (p.Y73*) - BBS5_000080 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
?/. 1 - c.226A>G r.(?) p.(Ile76Val) - VUS g.170344333A>G - BBS5(NM_152384.2):c.226A>G (p.I76V) - BBS5_000052 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 2 - c.258+2T>C r.spl, r.spl? p.? - pathogenic g.170344367T>C g.169487857T>C - - BBS5_000022 VKGL data sharing initiative Nederland PubMed: Haer-Wigman 2017 - - CLASSIFICATION record, Germline yes - - - - VKGL-NL_Nijmegen
-?/. 1 - c.259-3C>T r.spl? p.? - likely benign g.170344494C>T g.169487984C>T BBS5(NM_152384.2):c.259-3C>T - BBS5_000009 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/., +?/., -?/. 5 5 c.265C>T r.(?) p.(Arg89*), p.(Arg89Ter) - likely benign, likely pathogenic, pathogenic g.170344503C>T g.169487993C>T BBS5 p.R89X, IVS7-27T > G, BBS5(NM_152384.3):c.265C>T (p.R89*), p.R89X (c.C265T) - BBS5_000010 no c. position written in publication, probable position given - RCV001548296.2, 1 more item PubMed: Hirano 2015, PubMed: Hirano 2020 - - CLASSIFICATION record, Germline ? - - - - VKGL-NL_Utrecht
-/. 1 - c.285C>T r.(?) p.(Leu95=) - benign g.170344523C>T - BBS5(NM_152384.3):c.285C>T (p.L95=) - BBS5_000034 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-/. 1 - c.308G>A r.(?) p.(Ser103Asn) - benign g.170344546G>A g.169488036G>A BBS5(NM_152384.3):c.308G>A (p.S103N) - BBS5_000011 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
?/. 1 - c.365C>T r.(?) p.(Thr122Ile) - VUS g.170344603C>T - BBS5(NM_152384.3):c.365C>T (p.(Thr122Ile)) - BBS5_000081 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. 1 5i c.387-618del232 r.(?) p.? - likely pathogenic g.170348766del232 - [p.C91LfsX5];[p.Y469X] - BBS5_000061 - PubMed: Deveault-2011 - - Unknown - - - - - LOVD
-/., -?/. 2 - c.387-15_387-12del r.(=) p.(=) - benign, likely benign g.170349369_170349372del g.169492859_169492862del BBS5(NM_152384.3):c.387-15_387-12delTGTT - BBS5_000012 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht, VKGL-NL_AMC
-?/. 2 - c.387-4A>G r.spl? p.? - likely benign g.170349380A>G g.169492870A>G BBS5(NM_152384.2):c.387-4A>G, BBS5(NM_152384.3):c.387-4A>G - BBS5_000013 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam
+?/. 4 - c.412C>T r.(?) p.(Arg138Cys) - likely pathogenic g.170349409C>T g.169492899C>T 2:170349409C>T ENST00000295240.3:c.412C>T (Arg138Cys), BBS5 c.412C>T, p.Arg138Cys, 2 more items - BBS5_000028 compound heterozygous, heterozygous PubMed: Carss 2017, PubMed: Castro-Sanchez 2019, PubMed: Turro 2020 - - Germline, Germline/De novo (untested) ?, yes - - - - Sheila Castro-Sánchez
+/., ?/. 2 6 c.413G>A r.(?) p.(Arg138His) - pathogenic, VUS g.170349410G>A - c.471G>A, c.[413G>A];[413G>A] - BBS5_000054 - PubMed: Redin-2012, PubMed: Sathya Priya-2015 - - Germline no, yes - - - - LOVD
+?/. 2 - c.425T>A r.(?) p.(Leu142Ter) - likely pathogenic g.170349422T>A g.169492912T>A BBS5 L142X - BBS5_000069 homozygous PubMed: Li 2004 - - Germline yes - - - - LOVD
+/. 3 6 c.425T>G r.(?) p.(Leu142*), p.(Leu142Ter) - pathogenic g.170349422T>G g.169492912T>G BBS5(NM_152384.3):c.425T>G (p.L142*), p.*142Lext*? - BBS5_000014 VKGL data sharing initiative Nederland PubMed: Chandrasekar-2018 - - CLASSIFICATION record, Germline - - - - - VKGL-NL_Utrecht, VKGL-NL_VUmc
-?/. 1 - c.450G>A r.(?) p.(Lys150=) - likely benign g.170349447G>A - BBS5(NM_152384.2):c.450G>A (p.K150=) - BBS5_000053 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. 1 - c.501T>A r.(?) p.(Val167=) - benign g.170349498T>A - BBS5(NM_152384.3):c.501T>A (p.V167=) - BBS5_000015 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+?/. 2 - c.522+3A>G r.(?) p.? - likely pathogenic g.170349522A>G g.169493012A>G BBS5 A->G transition at the +3 position of the exon 6 splice donor - BBS5_000070 homozygous PubMed: Li 2004 - - Germline yes - - - - LOVD
-?/. 1 6i c.522+317A>G r.? p.? - likely benign g.170349836A>G g.169493326A>G - - BBS5_000048 - PubMed: Fadaie 2021 - - Germline - - - - - Zeinab Fadaie
+?/. 2 - c.543del r.(?) p.(Phe181Leufs*5) - likely pathogenic g.170350271del g.169493761del 538delT / g.914delT, c.538delT/p.(F180Ffs*6) - BBS5_000029 compound heterozygous PubMed: Castro-Sanchez 2019 - - Germline yes - - - - Sheila Castro-Sánchez
+/. 1 7 c.547A> G r.(?) p.(Thr183Ala) - pathogenic g.170350275A>G g.169493765A>G BBS5 c.547A> G, p.Thr183Ala - BBS5_000071 homozygous PubMed: Hjortshoj 2008 - - Germline yes 0/54 Indian individuals - - - LOVD
+/., +?/., -?/., ?/. 22 7 c.551A>G r.(?) p.(Asn184Ser) ACMG likely benign, likely pathogenic, pathogenic, VUS g.170350279A>G g.169493769A>G 2:170350279A>G ENST00000295240.3:c.551A>G (Asn184Ser), BBS5 c.551A>G, p.Asn184Ser, p.N184S, 10 more items - BBS5_000035 conflicting interpretations of pathogenicity; 3 heterozygous, no homozygous; Clinindb (India), 5 more items PubMed: Avela 2018, PubMed: Billingsley-2010, PubMed: Booij-2011, PubMed: Carss 2017, PubMed: Li 2004, 11 more items - rs137853921 CLASSIFICATION record, Germline, Germline/De novo (untested), Unknown ?, yes 3% ; absent in 96 controls, 3/2795 individuals - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Utrecht, VKGL-NL_AMC, Mohammed Faruq
?/. 1 - c.560T>C r.(?) p.(Ile187Thr) - VUS g.170350288T>C - BBS5(NM_152384.3):c.560T>C (p.(Ile187Thr)) - BBS5_000084 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 1 7 c.565C>T r.(?) p.? - pathogenic g.170350293C>T - c.[565C>T];[565C>T] - BBS5_000062 - PubMed: Redin-2012 - - Germline yes - - - - LOVD
?/. 1 - c.584A>G r.(?) p.(Asp195Gly) - VUS g.170350312A>G g.169493802A>G - - BBS5_000040 - PubMed: Lindstrand 2016 - - Germline - - - - - LOVD
?/. 1 - c.610C>A r.(?) p.(Leu204Met) - VUS g.170350338C>A - BBS5(NM_152384.3):c.610C>A (p.(Leu204Met)) - BBS5_000085 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-/. 1 - c.618+139T>C r.(?) p.(=) - benign g.170350485T>C g.169493975T>C - - BBS5_000074 - PubMed: Smaoui 2006 - - Germline - 2/19 families BBS - - - Johan den Dunnen
+/., +?/., -?/. 4 7i c.619-27T>G r.(=), r.spl? p.(=), p.(?) - likely benign, likely pathogenic, pathogenic g.170354110T>G g.169497600T>G BBS5 p.R89X, IVS7-27T > G, IVS7-27T>G - BBS5_000064 - PubMed: Hirano 2015, PubMed: Hirano 2020 - - Germline ? - - - - LOVD
-/. 1 - c.619-9T>C r.(?) p.(=) - benign g.170354128T>C g.169497618T>C - - BBS5_000075 - PubMed: Smaoui 2006 - - Germline - 1/19 families BBS - - - Johan den Dunnen
+/., +?/. 6 7i c.619-1G>C r.spl, r.spl? p.? ACMG likely pathogenic, pathogenic, pathogenic (recessive) g.170354136G>C g.169497626G>C BBS5(NM_152384.3):c.619-1G>C, c.619-1G>C - BBS5_000023 ACMG PM2, PVS1_STRONG, PP5_STRONG; no variant 2nd chromosome, unknown variant 2nd chromosome, 1 more item PubMed: Feuillan-2011, PubMed: Sharon 2019, PubMed: Weisschuh 2024 - - CLASSIFICATION record, Germline - 1/2420 IRD families - - - Global Variome, with Curator vacancy, Johan den Dunnen, VKGL-NL_Leiden, VKGL-NL_Nijmegen
-/., -?/., ?/. 3 - c.620G>A r.(?) p.(Arg207His) ACMG benign, likely benign, VUS g.170354138G>A g.169497628G>A BBS5(NM_152384.3):c.620G>A (p.R207H), BBS5, c.620G>A, p.Arg207His, Triallelism - BBS5_000036 VKGL data sharing initiative Nederland PubMed: Perea-Romero 2021 - - CLASSIFICATION record, Germline yes - - - - VKGL-NL_Utrecht, VKGL-NL_AMC
+?/. 1 - c.681+1G>T r.spl? p.? - likely pathogenic g.170354200G>T g.169497690G>T BBS5(NM_152384.3):c.681+1G>T - BBS5_000027 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-?/. 1 - c.681+19T>C r.(=) p.(=) - likely benign g.170354218T>C g.169497708T>C BBS5(NM_152384.2):c.681+19T>C - BBS5_000016 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 1 - c.682-801_*3862del r.? p.? - pathogenic g.170355195_170364954del - del exon8_12 170355189-170364951del - BBS5_000046 9,762 bp deletion, AluYc3 - AluSg PubMed: Lindstrand 2016 - - Germline - - - - - LOVD
-/. 1 - c.682-52A>T r.(?) p.(=) - benign g.170355944A>T g.169499434A>T - - BBS5_000076 - PubMed: Smaoui 2006 - rs12692909 Germline - 5/19 families BBS - - - Johan den Dunnen
+/., +?/. 3 - c.682-2A>G r.spl p.(?), p.? - likely pathogenic, pathogenic (recessive) g.170355994A>G g.169499484A>G BBS5* (NM_152384) (LOVD#0000638715), c.682-2A > G, Splice site,, g.20307A>G - BBS5_000039 homozygous PubMed: Gumus 2021 - - Germline yes - - - - Evren Gümüş
?/. 1 - c.708A>G r.(?) p.(Lys236=) - VUS g.170356022A>G g.169499512A>G BBS5(NM_152384.2):c.708A>G (p.K236=) - BBS5_000037 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/., +?/. 6 9 c.734_744del r.(?) p.(Asp245Glyfs*18), p.(Glu245Glyfs*18) - likely pathogenic (recessive), pathogenic g.170356048_170356058del g.169499538_169499548del BBS5 c.734_744del , p.(E245Gfs*18), g.25043_25053del - BBS5_000002 homozygous PubMed: Maria 2016 - - Germline yes - - - - Muhammad Ajmal
-/. 1 - c.750C>T r.(?) p.(Ile250=) - benign g.170356064C>T g.169499554C>T BBS5(NM_152384.3):c.750C>T (p.I250=) - BBS5_000038 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+?/., -?/., ?/. 5 8 c.751A>G r.(?) p.(Asn251Asp), p.(Lys251Glu) - likely benign, likely pathogenic, VUS g.170356065A>G g.169499555A>G BBS5(NM_152384.2):c.751A>G (p.N251D), BBS5(NM_152384.3):c.751A>G (p.N251D, p.(Asn251Asp)), 1 more item - BBS5_000017 VKGL data sharing initiative Nederland PubMed: Deveault-2011 - - CLASSIFICATION record, Unknown - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Utrecht, VKGL-NL_AMC
+/., +?/. 3 - c.790G>A r.(?) p.(Gly264Arg) - likely pathogenic, likely pathogenic (recessive), pathogenic g.170356104G>A g.169499594G>A BBS5, variant 1: c.790G>A/p.G264R, variant 2: c.790G>A/p.G264R - BBS5_000024 possibly solved, homozygous, VKGL data sharing initiative Nederland PubMed: Weisschuh 2016, PubMed: Weisschuh 2020 - - CLASSIFICATION record, Germline yes - - - - VKGL-NL_Nijmegen
+/. 1 - c.817-1G>C r.spl? p.? - pathogenic g.170359604G>C - BBS5(NM_152384.2):c.817-1G>C - BBS5_000041 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 11 c.889G>A r.(?) p.(Glu297Lys) - likely pathogenic g.170359677G>A - c.889G>A - BBS5_000063 unknown variant 2nd chromosome PubMed: Feuillan-2011 - - Germline - - - - - LOVD
+?/. 1 - c.900G>C r.(=) p.(=) - likely pathogenic g.170359688G>C - 2:170359688G>C ENST00000295240.3:c.900G>C (?)) - BBS5_000043 - PubMed: Carss 2017 - - Germline - - - - - LOVD
-/. 1 - c.901-49C>G r.(?) p.(=) - benign g.170360764C>G g.169504254C>G - - BBS5_000077 - PubMed: Smaoui 2006 - - Germline - 14/19 families BBS - - - Johan den Dunnen
+/. 2 - c.925_931del r.(?) p.(Gln309Ilefs*14) - pathogenic g.170360991_170360997del g.169504481_169504487del BBS5 c.925_931del, (p.Gln309Ilefs*14) - BBS5_000025 heterozygous PubMed: Torrefranca 2020 - - Germline yes - - - - LOVD
+?/. 1 - c.955_957del r.(?) p.(Glu319del) - likely pathogenic g.170361021_170361023del g.169504511_169504513del - - BBS5_000049 - PubMed: Patel 2016 - - Germline - - - - - LOVD
+?/. 8 12 c.966dup r.(?) p.(Ala323Cysfs*57), p.(Ala323CysfsTer57), p.(Ala323CysfsX57) - likely pathogenic g.170361032dup g.169504522dup BBS5 c.966dup, p.(Ala323Cysfs*57), BBS5 c.966dupT, p.(Ala323CysfsX57) - BBS5_000050 homozygous PubMed: Al-Hamed 2014, PubMed: Aldahmesh 2014, PubMed: Patel 2016 - - Germline ?, yes 0/100 normal controls from a Saudi Arabian population - - - LOVD
+?/. 1 - c.974A>G r.(?) p.(Glu325Gly) - likely pathogenic g.170361040A>G g.169504530A>G - - BBS5_000026 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-?/. 1 - c.*5204C>T r.(=) p.(=) - likely benign g.170366296C>T - KLHL41(NM_006063.2):c.8C>T (p.(Ser3Phe)) - KLHL41_000012 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.*5594G>A r.(=) p.(=) - likely benign g.170366686G>A - KLHL41(NM_006063.2):c.398G>A (p.(Gly133Asp)) - BBS5_000042 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.*5677C>T r.(=) p.(=) - VUS g.170366769C>T g.169510259C>T - - KLHL41_000003 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 - c.*5827_*5828del r.(=) p.(=) - VUS g.170366919_170366920del g.169510409_169510410del KLHL41(NM_006063.2):c.631_632del (p.(Arg211GlyfsTer2)) - KLHL41_000001 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.*5880A>C r.(=) p.(=) - likely benign g.170366972A>C - KLHL41(NM_006063.3):c.684A>C (p.T228=) - BBS5_000079 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
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