Unique variants in the BBS1 gene

This database is one of the "Eye disease" gene variant databases.
Information The variants shown are described using the NM_024649.4 transcript reference sequence.

214 entries on 3 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.(159+1_160-1)_(1110+1_1111-1) r.(?) p.(Leu54_Pro370del) - pathogenic (recessive) g.? g.? BBS1 c.(159+1_160-1)_(1110+1_1111-1), p.(Leu54_Pro370del) - NPHS1_000138 compound heterozygous PubMed: Delvallee 2021 - - Germline yes - - - - LOVD
+/. 1 - c.-3291_1111-1020del r.? p.? - pathogenic g.66274840_66292574del - del exon1_11 - DPP3_000008 17,734 bp deletion, AluY - AluY PubMed: Lindstrand 2016 - - Germline - - - - - LOVD
-/. 1 - c.-1555G>A r.(?) p.(=) - benign g.66276576G>A g.66509105G>A DPP3(NM_130443.3):c.2068G>A (p.E690K) - DPP3_000006 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 1 2i_12i c.(?_-12-1)_(1110+1_?)del r.(?) p.? - pathogenic g.(?_66278118)_(66291354_?)del - delexon1_11;het - BBS1_000219 - PubMed: Lindstrand-2014 - - Germline - - - - - LOVD
+/., +?/., ?/. 29 13, 16 c.? r.(?), r.0?, r.? p.(Asp278fs*9), p.(G318Vfs*61), p.(His504fs*48), p.0?, p.?, p.F534A, p.I296TfsX7, p.M242RfsX83 ACMG likely pathogenic, pathogenic, pathogenic (recessive), VUS g.66277761_66300761dup, g.66293652G>T, g.66298461_66298463del, g.? g.66510290_66533290dup c.[1169G>T];[1169G>T], C91fsX95/Y321X, D8D/R483X, L548fsX579, M390R/N524del†, p.(Asp278fs*9), 19 more items - DRD4_000002 normal 2nd chromosome PubMed: Billingsley-2010, PubMed: Carrigan 2016, PubMed: Castro Sanchez 2015, PubMed: Deveault-2011, 7 more items - - Germline, Unknown yes 0/96 ethnically matched controls - - - LOVD
+?/. 2 3 c.1A>G r.? p.? - likely pathogenic g.66278131A>G - M1V, [p.M390R];[p.L505PfsX52] - BBS1_000188 - PubMed: Deveault-2011, PubMed: Gerth-2008 - - Germline, Unknown - - - - - LOVD
-?/., ?/. 2 - c.19T>A r.(?) p.(Ser7Thr) - likely benign, VUS g.66278149T>A g.66510678T>A BBS1(NM_024649.5):c.19T>A (p.(Ser7Thr), p.S7T) - BBS1_000063 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Utrecht
+?/., -/., ?/. 5 3 c.24T>C r.(=), r.(?) p.(=), p.(Asp8=) - benign, likely pathogenic, VUS g.66278154T>C g.66510683T>C BBS1(NM_024649.5):c.24T>C (p.D8=), D8D/R483X, [p.M390R];[p.N524del] - BBS1_000064 VKGL data sharing initiative Nederland PubMed: Deveault-2011, PubMed: Gerth-2008, PubMed: Tiwari 2016 - - CLASSIFICATION record, Germline, Unknown - - - - - VKGL-NL_Utrecht, VKGL-NL_AMC
+/. 3 3 c.39del r.(?) p.(Ala14Profs*61) - pathogenic g.66278169del - c.39delA, c.[39del - BBS1_000173 - PubMed: M'hamdi 2014, PubMed: Redin-2012 - - Germline, Unknown yes - - - - LOVD
+?/. 1 - c.41C>G r.(?) p.(Ala14Gly) - likely pathogenic g.66278171C>G g.66510700C>G BBS1, variant 1: c.1169T>G/p.M390R, variant 2: c.41C>G/p.A14G - BBS1_000229 possibly solved, compound heterozygous PubMed: Weisschuh 2020 - - Unknown ? - - - - LOVD
+?/. 1 1 c.46A>T c.46A>T p.(Ser16Cys) ACMG likely pathogenic g.66278176A>T g.66510705A>T BBS1 c.46A>T, p.(Ser16Cys) - BBS1_000189 heterozygous PubMed: Manara 2019 - rs772917364 Germline ? - - - - LOVD
+?/. 1 - c.47G>C r.(?) p.(Ser16Thr) - likely pathogenic g.66278177G>C - BBS1(NM_024649.5):c.47G>C (p.S16T) - BBS1_000178 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
?/. 1 - c.48-9C>A r.(=) p.(=) - VUS g.66278475C>A g.66511004C>A BBS1(NM_024649.5):c.48-9C>A - BBS1_000111 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 1 - c.48-3C>G r.(?) p.? - pathogenic (recessive) g.66278481C>G g.66511010C>G - - BBS1_000166 - PubMed: Ece Solmaz 2015 - - Germline - - - - - LOVD
+?/. 1 - c.48-2A>C r.spl? p.? - likely pathogenic g.66278482A>C - BBS1(NM_024649.5):c.48-2A>C - BBS1_000262 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/., +?/. 2 2i c.48-1G>A r.spl? p.? - likely pathogenic, pathogenic g.66278483G>A - c.48-1G>A, IVS1-1g/a - BBS1_000171 - PubMed: Harville-2010, PubMed: Knopp 2015 - - Germline - 0/90 ethnically matched controls - - - LOVD
-/. 1 - c.57C>G r.(?) p.(Ala19=) - benign g.66278493C>G g.66511022C>G BBS1(NM_024649.5):c.57C>G (p.A19=) - BBS1_000112 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+?/. 1 - c.68G>A r.(?) p.(Trp23*) - likely pathogenic g.66278504G>A g.66511033G>A c.68G>A/p.(W23*) - BBS1_000185 homozygous PubMed: Alvarez-Satta 2014, PubMed: Castro-Sanchez 2019 - - Germline ? - - - - LOVD
?/. 1 - c.75T>A r.(?) p.(Asp25Glu) - VUS g.66278511T>A g.66511040T>A - - BBS1_000096 - PubMed: Koyanagi 2019, Journal: Koyanagi 2019 - rs772010847 Germline - 7/1204 cases with retinitis pigmentosa - - - Yoshito Koyanagi
+/. 3 - c.118del r.(?) p.(Cys40Alafs*2), p.(Cys40AlafsTer2) - pathogenic, pathogenic (recessive), VUS g.66278554del, g.68705674C>A g.66511083del, g.68938206C>A 118delT - IGHMBP2_000003 - PubMed: Bravo-Gil 2016, PubMed: Sanchez-Navarro 2018 - - Germline - - - - - Gerard C.P. Schaafsma
+/., +?/. 12 3i c.124+1G>A r.125_159del, r.spl? p.?, p.L43Gfs*44 - likely pathogenic, pathogenic g.66278561G>A - c.124+1G>A r.125_159del p.(L43Gfs*44), IVS2+1g/a, 1 more item - BBS1_000172 - PubMed: Abu-Safieh-2012, Abu-Safieh 2010, PubMed: Harville-2010 - - Germline - 0/90 ethnically matched controls, 0/96 ethnically matched controls - - - LOVD
+?/. 1 2 c.124+2T>G r.(?) p.(Asn17Alafs*56) - likely pathogenic (recessive) g.66278562T>G g.66511091T>G BBS1 c.124+2T>G, p.N17AfsX56 - BBS1_000244 compound heterozygous; exon 2 skipping PubMed: Katagiri 2020 - - Germline yes - - - - LOVD
-?/. 1 - c.124+17A>G r.(=) p.(=) - likely benign g.66278577A>G - BBS1(NM_024649.5):c.124+17A>G - BBS1_000250 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 1 2i_6i c.(124+1_125-1)_(518+1_519-1)del r.? p.? - pathogenic g.(66278561_66278675)_(66283203_66283331)del g.(66511090_66511204)_(66515732_66515860)del del ex3-6 - chr11_008601 - PubMed: Retterer 2016 - - Unknown - - - - - Johan den Dunnen
+?/. 1 - c.159G>C r.(?) p.(Lys53Asn) - VUS g.66278710G>C - - - BBS1_000170 - - - - Germline/De novo (untested) - - - - - Jinu Han
?/. 1 - c.163G>A r.(?) p.(Val55Met) - VUS g.66281880G>A g.66514409G>A - - BBS1_000097 - PubMed: Koyanagi 2019, Journal: Koyanagi 2019 - rs181765153 Germline - 5/1204 cases with retinitis pigmentosa - - - Yoshito Koyanagi
?/. 1 - c.175C>G r.(?) p.(Leu59Val) - VUS g.66281892C>G - BBS1(NM_024649.5):c.175C>G (p.(Leu59Val)) - BBS1_000259 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.194A>G r.(?) p.(Gln65Arg) - VUS g.66281911A>G - BBS1(NM_024649.5):c.194A>G (p.(Gln65Arg)) - BBS1_000263 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 1 4 c.217G>T r.(?) p.(Gly73*) - pathogenic g.66281934G>T - c.217G>T - BBS1_000190 - PubMed: Muller-2010 - - Germline - - - - - LOVD
+/., +?/. 3 4 c.223_224del r.(?) p.(Leu75Glyfs*23) - likely pathogenic, pathogenic g.66281940_66281941del g.66514469_66514470del 223_224delCT, c.223_224delCT(h) - BBS1_000153 - PubMed: Janssen-2011, PubMed: Stone 2017 - - Germline - 0.009 - - - LOVD
-?/., ?/. 5 - c.235G>A r.(?) p.(Glu79Lys) - likely benign, VUS g.66281952G>A g.66514481G>A BBS1(NM_024649.4):c.235G>A (p.E79K), BBS1(NM_024649.5):c.235G>A (p.E79K, p.(Glu79Lys)) - BBS1_000113 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Utrecht, VKGL-NL_AMC
-?/. 2 - c.243G>A r.(?) p.(Pro81=) - likely benign g.66281960G>A g.66514489G>A BBS1(NM_024649.5):c.243G>A (p.P81=) - BBS1_000070 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht, VKGL-NL_Nijmegen
?/. 1 - c.299G>A r.(?) p.(Arg100Gln) - VUS g.66282016G>A - BBS1(NM_024649.5):c.299G>A (p.(Arg100Gln)) - BBS1_000264 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-/., -?/. 2 - c.316C>G r.(?) p.(Leu106Val) - benign, likely benign g.66282033C>G g.66514562C>G BBS1(NM_024649.4):c.316C>G (p.L106V), BBS1(NM_024649.5):c.316C>G (p.L106V) - BBS1_000065 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Utrecht
+/. 1 6 c.320_332dup r.(?) p.(Val112Phefs*9) - pathogenic g.66282037_66282049dup - 320-332Dup13pb - BBS1_000191 - PubMed: Muller-2010 - - Germline - - - - - LOVD
+?/., ?/. 3 4 c.329C>A r.(?) p.(Pro110His) - likely pathogenic, VUS g.66282046C>A g.66514575C>A BBS1 c.329C>A, p.Pro110His, C329A - BBS1_000098 heterozygous PubMed: Katagiri 2014, PubMed: Koyanagi 2019, Journal: Koyanagi 2019, PubMed: Liu 2020 - rs750288768 Germline, Germline/De novo (untested) ? 1/64, 2/1204 cases with retinitis pigmentosa - - - Yoshito Koyanagi
-?/. 1 - c.360C>T r.(?) p.(Pro120=) - likely benign g.66282077C>T g.66514606C>T BBS1(NM_024649.4):c.360C>T (p.P120=) - BBS1_000066 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 2 - c.363C>A r.(?) p.(Tyr121Ter) - pathogenic g.66282080C>A g.66514609C>A BBS1(NM_024649.5):c.363C>A (p.Y121*) - BBS1_000067 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht, VKGL-NL_Nijmegen
-/., ?/. 5 4 c.378G>A r.(=), r.(?) p.(=), p.(Leu126=) - benign, VUS g.66282095G>A g.66514624G>A BBS1(NM_024649.4):c.378G>A (p.L126=), BBS1(NM_024649.5):c.378G>A (p.L126=) - BBS1_000061 VKGL data sharing initiative Nederland PubMed: Almomani 2011 {dbSNP:2298806}, PubMed: Smaoui 2006 - rs2298806 CLASSIFICATION record, Germline - 6/19 families BBS - - - Gerard C.P. Schaafsma, Johan den Dunnen, VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Nijmegen
+?/. 1 7 c.382C>T r.(?) p.(Gln128*) - likely pathogenic g.66282099C>T - c.382C>T - BBS1_000192 - PubMed: Muller-2010, Beales 2003 - - Germline - - - - - LOVD
+/. 1 - c.432+1G>A r.spl p.(?) - pathogenic g.66282150G>A g.66514679G>A BBS1 c.432+1G>A - BBS1_000222 heterozygous PubMed: Zampaglione 2020 - - Unknown ? - - - - LOVD
-?/. 1 - c.432+13C>T r.(=) p.(=) - likely benign g.66282162C>T - BBS1(NM_024649.5):c.432+13C>T - BBS1_000251 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-?/. 1 - c.433-5C>T r.spl? p.? - likely benign g.66283006C>T g.66515535C>T BBS1(NM_024649.4):c.433-5C>T (p.?) - BBS1_000114 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.433-3C>T r.spl? p.? - likely benign g.66283008C>T g.66515537C>T BBS1(NM_024649.4):c.433-3C>T (p.?) - BBS1_000115 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/., +?/. 15 7 c.436C>T r.(?) p.(Arg146*), p.(Arg146Ter), p.? - likely pathogenic, pathogenic, pathogenic (recessive) g.66283014C>T g.66515543C>T 436C>T, 448C>T (Arg146Stop), BBS1(NM_024649.5):c.436C>T (p.R146*), c.436C>T, p.(Arg146*), 4 more items - BBS1_000116 solved, compound heterozygous, VKGL data sharing initiative Nederland, 1 more item PubMed: Castro Sanchez 2015, PubMed: Fauser-2003, Beales 2003, PubMed: Feuillan-2011, 7 more items - - CLASSIFICATION record, Germline, Unknown ?, yes 0.009, 0.015 - - - Johan den Dunnen, VKGL-NL_AMC
?/. 1 - c.437G>A r.(?) p.(Arg146Gln) - VUS g.66283015G>A g.66515544G>A - - BBS1_000099 - PubMed: Koyanagi 2019, Journal: Koyanagi 2019 - rs759253107 Germline - 1/1204 cases with retinitis pigmentosa - - - Yoshito Koyanagi
?/. 1 - c.437G>C r.(?) p.(Arg146Pro) - VUS g.66283015G>C g.66515544G>C - - BBS1_000258 - - - - Germline - - - - - Tamar Ben-Yosef
+/. 1 7 c.442G>A r.(?) p.(Glu148Lys) - pathogenic g.66283020G>A - c.442G>A - BBS1_000193 - PubMed: Hoskins-2003 - - Germline - - - - - LOVD
+/. 3 7 c.448C>T r.(?) p.? - pathogenic (recessive) g.66283026C>T - c.448C>T - DRD4_000002 - PubMed: M'hamdi-2014 - - Germline - - - - - LOVD
?/. 1 - c.472A>G r.(?) p.(Ser158Gly) - VUS g.66283050A>G g.66515579A>G - - BBS1_000100 - PubMed: Koyanagi 2019, Journal: Koyanagi 2019 - - Germline - 1/1204 cases with retinitis pigmentosa - - - Yoshito Koyanagi
?/. 1 - c.478C>T r.(?) p.(Arg160Trp) - VUS g.66283056C>T g.66515585C>T - - BBS1_000141 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/., +?/., ?/. 13 5, 6, 7 c.479G>A r.(?) p.(Arg160Gln), p.? ACMG likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS g.66283057G>A g.66515586G>A 11:66283057G>A ENST00000318312.7:c.479G>A (Arg160Gln), BBS1(NM_024649.4):c.479G>A (p.R160Q), 5 more items - BBS1_000071 ACMG PP3, PM2, PP5_STRONG, Homozygous, normal 2nd chromosome, solved, homozygous, 1 more item PubMed: Carss 2017, PubMed: Deveault-2011, PubMed: Duelund Hjortshoj-2010, PubMed: Hichri-2005, 6 more items 370228 - CLASSIFICATION record, Germline, Unknown yes 2/2420 IRD families - - - Global Variome, with Curator vacancy, Johan den Dunnen, Dror Sharon, VKGL-NL_Rotterdam, VKGL-NL_Nijmegen
-/. 1 - c.479+20T>C r.(=) p.(=) - benign g.66283077T>C g.66515606T>C BBS1(NM_024649.5):c.479+20T>C - BBS1_000069 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 2 5i c.480-1G>C r.spl? p.? - pathogenic g.66283163G>C g.66515692G>C BBS1(NM_024649.5):c.480-1G>C, c.IVS5-1G>C(H) - BBS1_000135 VKGL data sharing initiative Nederland PubMed: Janssen-2011 - - CLASSIFICATION record, Germline - 0.037 - - - VKGL-NL_AMC
+/. 1 8 c.508C>T r.(?) p.(Gln170*) - pathogenic g.66283192C>T - c.508C>T - BBS1_000194 - PubMed: Muller-2010 - - Germline - - - - - LOVD
+?/. 1 8i c.518+1G>A r.(?) p.? - VUS g.66283203G>A - c.518+1G>A - BBS1_000206 - PubMed: Chen-2011 - - Germline - - - - - LOVD
-?/. 1 - c.519-20C>G r.(=) p.(=) - likely benign g.66283312C>G - BBS1(NM_024649.5):c.519-20C>G - BBS1_000252 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+?/. 1 6i c.519-2A>G r.(?) p.(?) - likely pathogenic g.66283330A>G g.66515859A>G BBS1 IVS6 c.519-2A>G p.(?), Ex.12 c.1169T>G p.(Met390Arg) - BBS1_000195 compound heterozygous PubMed: Martin Merida 2019 - - Germline/De novo (untested) ? - - - - LOVD
+?/. 1 - c.526C>T r.(?) p.(Gln176Ter) ACMG likely pathogenic (recessive) g.66283339C>T g.66515868C>T - - BBS1_000254 ACMG PM2, PVS1 PubMed: Weisschuh 2024 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.533A>G r.(?) p.(Glu178Gly) - VUS g.66283346A>G g.66515875A>G BBS1(NM_024649.5):c.533A>G (p.E178G) - BBS1_000078 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. 1 - c.564A>C r.(?) p.(Gln188His) - VUS g.66283377A>C g.66515906A>C - - BBS1_000101 - PubMed: Koyanagi 2019, Journal: Koyanagi 2019 - - Germline - 1/1204 cases with retinitis pigmentosa - - - Yoshito Koyanagi
?/. 1 - c.566A>G r.(?) p.(His189Arg) - VUS g.66283379A>G g.66515908A>G - - BBS1_000102 - PubMed: Koyanagi 2019, Journal: Koyanagi 2019 - rs749036245 Germline - 1/1204 cases with retinitis pigmentosa - - - Yoshito Koyanagi
?/. 1 - c.567C>G r.(?) p.(His189Gln) - VUS g.66283380C>G g.66515909C>G - - BBS1_000103 - PubMed: Koyanagi 2019, Journal: Koyanagi 2019 - rs768596720 Germline - 1/1204 cases with retinitis pigmentosa - - - Yoshito Koyanagi
+/. 2 - c.589C>T r.(?) p.(Gln197Ter) - pathogenic g.66283402C>T g.66515931C>T BBS1(NM_024649.4):c.589C>T (p.Q197*), BBS1(NM_024649.5):c.589C>T (p.Q197*) - BBS1_000079 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_AMC
-?/. 1 - c.591+9C>G r.(=) p.(=) - likely benign g.66283413C>G g.66515942C>G BBS1(NM_024649.5):c.591+9C>G - BBS1_000080 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+/. 1 7i c.592-59G>A r.spl? p.? - pathogenic g.66287029G>A - c.592-59G>A/N - BBS1_000224 normal 2nd chromosome PubMed: Esposito 2017 - - Germline - - - - - LOVD
+/., +?/. 5 8 c.592-21A>T r.(=), r.[(592_621del,592_723del,=)] p.(=), p.[(Thr198_Lys207del,Thr198_Lys241del,=)] - likely pathogenic, pathogenic (recessive) g.66287067A>T g.66519596A>T - - BBS1_000165 effect on splicing predicted from minigene splicing assay, VKGL data sharing initiative Nederland PubMed: Fadaie 2022 - - CLASSIFICATION record, Germline yes - - - - VKGL-NL_Nijmegen, Zeinab Fadaie
+/. 2 7i_10i c.592-1_830+1del r.spl? p.? - pathogenic (recessive) g.66287087_66288848del - c.[592-?_830+?del];[436C>T] - BBS1_000235 - PubMed: Mary-2019 - - Germline - - - - - LOVD
+?/. 1 - c.599_604del r.(?) p.(Ile200_Thr201del) - likely pathogenic g.66287095_66287100del g.66519624_66519629del 597_602delCATCAC - BBS1_000154 - PubMed: Stone 2017 - - Germline - - - - - LOVD
-?/., ?/. 3 9 c.616T>G r.(?) p.(Leu206Val) - likely benign, VUS g.66287112T>G g.66519641T>G BBS1(NM_024649.5):c.616T>G (p.L206V), c.616T>G(h) - BBS1_000117 VKGL data sharing initiative Nederland PubMed: Janssen-2011 - - CLASSIFICATION record, Germline - - - - - VKGL-NL_Utrecht
+/. 2 9 c.664G>C r.(?) p.(Val222Leu) - pathogenic g.66287160G>C - c.664G>C/c.664G>C (p.G222R) - BBS1_000225 - PubMed: Esposito 2017 - - Germline - - - - - LOVD
+/., ?/. 4 9 c.670G>A r.(?) p.(Glu224Lys), p.? ACMG pathogenic, VUS g.66287166G>A g.66519695G>A c.[670G>A];[670G>A] - BBS1_000140 1 heterozygous, no homozygous; Clinindb (India), ACMG PP3, PM2, PP5 PubMed: Narang 2020, Journal: Narang 2020, PubMed: Redin-2012, PubMed: Weisschuh 2024 - rs193922709 Germline - 1/2794 individuals - - - Johan den Dunnen, Mohammed Faruq
+?/., -/., -?/., ?/. 7 9 c.700G>A r.(?) p.(Glu234Lys) - benign, likely benign, likely pathogenic, VUS g.66287196G>A g.66519725G>A BBS1(NM_024649.4):c.700G>A (p.E234K, p.(Glu234Lys)), BBS1(NM_024649.5):c.700G>A (p.E234K), E234K - BBS1_000118 4 heterozygous, no homozygous; Clinindb (India), VKGL data sharing initiative Nederland PubMed: Bryant 2018, PubMed: Eichers-2009, Badano 2003, PubMed: Narang 2020, Journal: Narang 2020 - rs35520756 CLASSIFICATION record, Germline - 4/2793 individuals - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Utrecht, VKGL-NL_AMC, Mohammed Faruq
+?/. 1 8 c.723+2T>G r.(?) p.(Thr198_Lys241del) - likely pathogenic (recessive) g.66287221T>G g.66519750T>G BBS1 c.723+2T>G, p.T198_K241del - BBS1_000245 compound heterozygous; exon 8 skipping PubMed: Katagiri 2020 - - Germline yes - - - - LOVD
+/. 1 - c.724-358_1165del r.? p.? - pathogenic g.66288383_66293648del - del exon9_12 66288383-66293649del - BBS1_000158 5,266 bp deletion, 1 bp microhomology (T) PubMed: Lindstrand 2016 - - Germline - - - - - LOVD
-/. 1 - c.724-17G>T r.(=) p.(=) - benign g.66288724G>T g.66521253G>T BBS1(NM_024649.5):c.724-17G>T - BBS1_000119 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-?/. 1 - c.724-16C>T r.(=) p.(=) - likely benign g.66288725C>T g.66521254C>T BBS1(NM_024649.5):c.724-16C>T - BBS1_000136 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/. 1 - c.724-13T>G r.(=) p.(=) - benign g.66288728T>G g.66521257T>G BBS1(NM_024649.5):c.724-13T>G - BBS1_000120 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/. 4 - c.724-8G>C r.(=) p.(=) - benign g.66288733G>C g.66521262G>C BBS1(NM_024649.4):c.724-8G>C, BBS1(NM_024649.5):c.724-8G>C - BBS1_000072 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Utrecht, VKGL-NL_Nijmegen
+?/., -?/. 2 10 c.734C>T r.(?) p.(Pro245Leu) - likely benign, likely pathogenic g.66288751C>T - BBS1(NM_024649.5):c.734C>T (p.P245L), c.734C>T(h) - BBS1_000220 unknown variant 2nd chromosome, VKGL data sharing initiative Nederland PubMed: Janssen-2011 - - CLASSIFICATION record, Germline - 1.6% ; absent in 96 controls - - - VKGL-NL_Utrecht
-/. 1 - c.736-8G>C r.(?) p.(=) - benign g.66288745G>C g.66521274G>C - - BBS1_000248 - PubMed: Smaoui 2006 - rs10896125 Germline - 2/19 families BBS - - - Johan den Dunnen
?/. 2 - c.742C>A r.(?) p.(Pro248Thr) - VUS g.66288759C>A - BBS1(NM_024649.4):c.742C>A (p.P248T), BBS1(NM_024649.5):c.742C>A (p.P248T) - BBS1_000121 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_AMC
-/. 1 - c.744C>T r.(?) p.(Pro248=) - benign g.66288761C>T g.66521290C>T BBS1(NM_024649.5):c.744C>T (p.P248=) - BBS1_000122 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. 2 - c.751C>G r.(?) p.(Leu251Val) - VUS g.66288768C>G - BBS1(NM_024649.4):c.751C>G (p.L251V), BBS1(NM_024649.5):c.751C>G (p.(Leu251Val)) - BBS1_000148 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam
+/. 1 - c.767del r.(?) p.(Gln256Argfs*20) ACMG pathogenic g.66288784del - c.767delA - BBS1_000143 - PubMed: Sharon 2019 - - Germline - 1/2420 IRD families - - - Global Variome, with Curator vacancy
?/. 1 - c.785G>A r.(?) p.(Arg262Gln) - VUS g.66288802G>A g.66521331G>A - - BBS1_000104 - PubMed: Koyanagi 2019, Journal: Koyanagi 2019 - rs747282202 Germline - 3/1204 cases with retinitis pigmentosa - - - Yoshito Koyanagi
?/. 1 - c.785G>C r.(?) p.(Arg262Pro) - VUS g.66288802G>C - BBS1(NM_024649.5):c.785G>C (p.(Arg262Pro)) - BBS1_000260 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 2 - c.815T>C r.(?) p.(Ile272Thr) - VUS g.66288832T>C g.66521361T>C BBS1 c.815T>C, p.Ile272Thr, c.815T>C, p.Ile272Thr - BBS1_000223 heterozygous, homozygous PubMed: Zampaglione 2020 - - Unknown ? - - - - LOVD
+/., +?/. 3 10 c.(830G>A), c.830G>A r.(?) p.(Arg277Lys), p.(Gly277Glu) - likely pathogenic, pathogenic g.66288847G>A g.66521376G>A BBS1 p.R277K, c.830G>A - BBS1_000207 no nucleotide annotation, extrapolated from protein and databases; heterozygous PubMed: Feuillan-2011, PubMed: Jacobson 2014, PubMed: Panneman 2023 - - Germline, Unknown ? - - - - Daan Panneman
+/. 1 10i c.830+1G>A r.spl? p.? - pathogenic g.66288848G>A - IVS9+1G>A - BBS1_000196 - PubMed: Fauser-2003 - - Unknown - - - - - LOVD
+/. 1 - c.831-797_*5514del r.0? p.0? - pathogenic g.66290130_66305022del - del exon10_17 - BBS1_000160 14,892 bp deletion PubMed: Lindstrand 2016 - - Germline - - - - - LOVD
-?/. 2 - c.831-5C>T r.spl? p.? - likely benign g.66290922C>T g.66523451C>T BBS1(NM_024649.4):c.831-5C>T (p.?) - BBS1_000123 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam
+?/. 1 - c.(830+1_831-1)_(1180+1_1181-1)del r.? p.(Asp278Trpfs*20) - pathogenic g.(66288848_66290926)_(66293664_66294119)del g.(66521377_66523455)_(66526193_66526648)del 831-?_1180+?del - BBS1_000174 - - - - Germline/De novo (untested) - - - - - Jinu Han
+?/. 1 - c.831_1110del r.(?) p.(Asp278Metfs*3) - likely pathogenic g.66290927_66291353del g.66523456_66523882del BBS1 Exon 10-11del, p.R429X - BBS1_000226 1 more item PubMed: Hirano 2020 - - Germline ? - - - - LOVD
+?/. 1 10 c.837del r.(?) p.(Arg280Alafs*18) - likely pathogenic g.66290933del - c.836delC/p.(Asp278fs*9)b - BBS1_000221 - PubMed: Alvarez-Satta-2014 - - Germline - 0/100 chromosomes - - - LOVD
+/. 2 10 c.851del r.(?) p.(Asp284Alafs*14), p.(Tyr284SerfsTer5) - pathogenic, pathogenic (recessive) g.66290947del g.66523476del 851delA, c.851delA - BBS1_000167 - PubMed: Ece Solmaz 2015, PubMed: Hoskins-2003 - - Germline - - - - - LOVD
+/., +?/. 3 10 c.863T>G r.(?) p.(Leu288Arg) - likely pathogenic, pathogenic g.66290959T>G - c.863T>G, c.863T>G/p.(Leu288Arg), p.(Leu288Arg) - BBS1_000197 - PubMed: Alvarez-Satta-2014, PubMed: Castro Sanchez 2015, PubMed: Muller-2010 - - Germline - - - - - LOVD
+/., +?/. 3 10 c.871C>T r.(?) p.(Gln291*) - likely pathogenic, pathogenic g.66290967C>T - BBS1(NM_024649.4):c.871C>T (p.Q291*, p.(Gln291*)), c.871C>T(h) - BBS1_000149 unknown variant 2nd chromosome, VKGL data sharing initiative Nederland PubMed: Janssen-2011 - - CLASSIFICATION record, Germline - 2.9% ; absent in 96 controls - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam
?/. 1 - c.887T>C r.(?) p.(Ile296Thr) - VUS g.66290983T>C g.66523512T>C BBS1(NM_024649.5):c.887T>C (p.I296T) - BBS1_000081 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. 1 11 c.889C>T r.(?) p.(Arg297Trp) - VUS g.66290985C>T - c.889C>T - BBS1_000208 - PubMed: Abu-Safieh-2012 - - Germline - - - - - LOVD
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