Unique variants in the BBS10 gene

This database is one of the "Eye disease" gene variant databases.
Information The variants shown are described using the NM_024685.3 transcript reference sequence.

160 entries on 2 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 1 c.-52C>T r.(=) p.(=) - VUS g.76742190G>A - c.-52C>T - BBS10_000188 - PubMed: Abu-Safieh-2012, Abu-Safieh 2010 - - Germline - - - - - LOVD
+/., +?/., ?/. 60 2 c.? r.(?) p.(Cys91fs*4), p.(Glu337del), p.(Leu533Leufs*21), p.(Tyr503fs*17), p.(Tyr559*), p.(Tyr559fs*17), p.?, 9 more items - likely pathogenic, pathogenic, VUS g.? g.? (c.157-3C>G)+(c.157-3C>G), c.1090del>A, c.272 insertion T, c.687delA(h), C91fsX95/C91fsX95, 35 more items - ALX1_000001 no real mutation annotation, most probably a known c.271dup, p.(Cys91Leufs*5) variant; homozygous, 2 more items PubMed: Billingsley-2010, PubMed: Castro Sanchez 2015, PubMed: Chen-2011, PubMed: Deveault-2011, 10 more items - - Germline, Unknown yes 0/96 ethnically matched controls, 50 control individuals - - - LOVD
+/. 1 1 c.9_14del r.(?) p.(Ser3_Met5delinsArg) - pathogenic g.76742125_76742130del - c.9_14delTTCTAT(p.Ser3_Met5delinsArg);het - BBS10_000199 - PubMed: Lindstrand-2014 - - Germline - - - - - LOVD
+/. 2 1 c.9_15delinsGC r.(?) p.(Ser3Argfs*91) - pathogenic g.76742124_76742130delinsGC g.76348344_76348350delinsGC c.9_15delinsGC(p.S3Rfs*91) - BBS10_000116 - PubMed: Wang 2016, PubMed: Zenteno 2020 - - Germline - 1/143 cases - - - Johan den Dunnen
+?/. 1 1 c.32T>G r.(?) p.(Val11Gly) - likely pathogenic g.76742107A>C - S311A/V11G - BBS10_000139 - PubMed: Laurier-2006 - - Germline yes 0/107 Lebanese controls; 0/96 French controls - - - LOVD
+?/. 1 - c.39_46del r.(?) p.(Ala14Glyfs*79) - likely pathogenic g.76742095_76742102del g.76348315_76348322del BBS10 c.39_46delGGCGTTGC - BBS10_000222 homozygous PubMed: Ladino 2018 - - Germline yes - - - - LOVD
+?/. 1 1 c.92C>T r.(?) p.(Pro31Leu) - likely pathogenic g.76742047G>A - c.92C>T - BBS10_000187 - PubMed: Feuillan-2011 - - Germline - - - - - LOVD
+/., +?/. 2 - c.98G>A r.(?) p.(Gly33Glu) - likely pathogenic, pathogenic g.76742041C>T g.76348261C>T BBS10 c.98G>A, p.(G33E), BBS10 p.G33E, p.R709X - BBS10_000205 heterozygous, no c. position written in publication, probable position given - c.98G>A PubMed: Hirano 2020, PubMed: Kurata 2018 - - Germline ?, yes - - - - LOVD
?/. 1 - c.101G>C r.(?) p.(Arg34Pro) - VUS g.76742038C>G - BBS10(NM_024685.4):c.101G>C (p.R34P) - BBS10_000217 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.124A>T r.(?) p.(Thr42Ser) - VUS g.76742015T>A g.76348235T>A BBS10(NM_024685.4):c.124A>T (p.T42S) - BBS10_000109 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 1 1 c.128A>G r.(?) p.? - pathogenic g.76742011T>C - c.128A>G - BBS10_000158 A not found at position given, found G instead. PubMed: Muller-2010 - - Germline - - - - - LOVD
+/., +?/., ?/. 34 1 c.145C>T r.(?) p.(Arg49Trp) ACMG likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS g.76741994G>A g.76348214G>A BBS10 c.145C>T, p.(Arg49Trp), BBS10 c.145C>T, p.(Arg49Trp), c.145C>T, c.145C>T (p.R49W), 10 more items - BBS10_000097 ACMG PM2, PP5_STRONG, compound heterozygous, heterozygous, homozygous, normal 2nd chromosome, 2 more items PubMed: Billingsley-2010, PubMed: Chen-2011, PubMed: Delvallee 2021, PubMed: Deveault-2011, 13 more items 225010 - CLASSIFICATION record, Germline, Unknown ?, yes - - - - Johan den Dunnen, VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Utrecht, VKGL-NL_Nijmegen, VKGL-NL_VUmc
+/., +?/. 7 1 c.164T>C r.(?) p.(Leu55Pro) - likely pathogenic, pathogenic, VUS g.76741975A>G g.76348195A>G BBS10(NM_024685.4):c.164T>C (p.L55P), c.164T>C, [L55P]+[E274VfsX29], [p.R413X];[p.R413X] - BBS10_000096 VKGL data sharing initiative Nederland PubMed: Billingsley-2010, PubMed: Chen-2011, PubMed: Deveault-2011 - - CLASSIFICATION record, Germline, Unknown - - - - - VKGL-NL_Rotterdam, VKGL-NL_Utrecht, VKGL-NL_VUmc, VKGL-NL_AMC
+?/. 1 1 c.180dup r.(?) p.(Glu61Argfs*35) - likely pathogenic g.76741959dup - c.180dupA - BBS10_000186 - PubMed: Feuillan-2011 - - Germline - - - - - LOVD
+?/. 1 - c.185A>G r.(?) p.(His62Arg) - likely pathogenic g.76741954T>C g.76348174T>C BBS10 c.185A>G, p.His62Arg - BBS10_000221 heterozygous PubMed: Putoux 2010 - - Germline yes - - - - LOVD
+?/. 1 1i c.197+1G>T r.spl? p.? - likely pathogenic g.76741941C>A - [p.K188T; p.A636V];[p.K188T; p.A636V] - BBS10_000185 - PubMed: Deveault-2011 - - Unknown - - - - - LOVD
-?/. 1 - c.198-3C>T r.spl? p.? - likely benign g.76741570G>A g.76347790G>A BBS10(NM_024685.4):c.198-3C>T - BBS10_000095 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+/. 2 2 c.198G>T r.(?) p.(Arg66Ser) - pathogenic (recessive) g.76741567C>A - c.[198G>T];[198G>T] - BBS10_000214 - PubMed: Mary-2019 - - Germline - - - - - LOVD
+?/. 2 2 c.219_229del r.(?) p.(Ser74Lysfs*18) - likely pathogenic g.76741536_76741546del - c.219delCAGTCATCTCA - BBS10_000157 - PubMed: Muller-2010, PubMed: Stoetzel 2006 - - Germline - - - - - LOVD
+/. 1 - c.233G>A r.(?) p.(Cys78Tyr) - pathogenic g.94768015G>A g.93755787G>A TMEM67 c.233G>A, p.(Cys78Tyr) - TMEM67_000217 heterozygous PubMed: Barabino 2020 - - In vitro (cloned) ? - - - - LOVD
+/., +?/. 3 2 c.235dup r.(?) p.(Thr79Asnfs*17) ACMG likely pathogenic, pathogenic g.76741530dup, g.76741536dup g.76347756dup 235dupA, BBS10 c.235dup, p.Thr79Asnfs*17, c.235dupA/c.271dupT (T79Nfs*17/p.C91LfsX5) - BBS10_000129 heterozygous PubMed: Barrell 2019, PubMed: Esposito 2017 - - Germline, Germline/De novo (untested), In vitro (cloned) ? - - - - Jinu Han
+/. 1 - c.270_271insT r.(?) p.(Cys91Leufs*5) - pathogenic (recessive) g.76741494_76741495insA - 12:76741493C>CA ENST00000393262.3:c.271dupT (Cys91LeufsTer5) - BBS10_000118 - PubMed: Carss 2017 - - Germline - - - - - LOVD
+/., +?/., ?/. 76 2 c.271dup c.271dup, r.(?) p.(Cys91Leufs*5), p.(Cys91LeufsTer5) ACMG likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS g.76741494dup, g.76741496dup g.76347714dup, g.76347716dup 268_269insT, 271dupT, BBS10 c.271dup, p.(Cys91Leufs*5), BBS10 c.271dupT, p.Cys91LeufsTer5, c.271dupT, 12 more items - BBS10_000002, BBS10_000092 ACMG PM2, PVS1_STRONG, PP5, PS4, founder mutation, heterozygous, homozygous, solved, homozygous, 4 more items PubMed: Alvarez-Satta-2014, PubMed: Barabino 2020, PubMed: Duelund Hjortshoj-2010, PubMed: Holtan 2020, 18 more items - rs549625604 CLASSIFICATION record, Germline, Germline/De novo (untested), In vitro (cloned), Unknown ?, yes 1/899 cases, 6/899 cases - - - Global Variome, with Curator vacancy, Johan den Dunnen, Karen Stals, VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Utrecht, VKGL-NL_Nijmegen, VKGL-NL_VUmc, VKGL-NL_AMC
+?/., ?/. 2 2 c.271dupG r.(?) p.? - likely pathogenic, VUS g.76741494dupC - (c.271dupG; p.Cys91fs), R49W/L414S - BBS10_000184 - PubMed: Dulfer-2010 - - Unknown - - - - - LOVD
+/., +?/. 8 - c.271dupT r.(?) p.(Cys91Leufs*5) - likely pathogenic, pathogenic, pathogenic (recessive) g.76741496dup g.76347716dup BBS10 c.271dupT, p.(Cys91Leufs*5), BBS10 c.271dupT, p.Cys91LeufsX5, BBS10 c.271insT, p.(Cys91fsX95) - BBS10_000001, BBS10_000002 compound heterozygous, father's DNA unavaiable; heterozygous, heterozygous, homozygous PubMed: Brunbjerg-Hey 2018, PubMed: Delvallee 2021, PubMed: Paolacci 2019, PubMed: Putoux 2010 - - Germline, Germline/De novo (untested), In vitro (cloned) ?, yes - - - - LOVD
+?/. 2 2 c.271_272insT r.(?) p.(Cys91Leufs*5) - likely pathogenic g.76741493_76741494insA - c.271_272insT - BBS10_000183 - PubMed: Schaefer-2011 - - Germline yes - - - - LOVD
+/. 1 - c.272dup r.(?) p.(Cys91Trpfs*5) - pathogenic g.76741493dup g.76347714dup - - BBS10_000110 1 more item PubMed: Narang 2020, Journal: Narang 2020 - rs549625604 Germline - 3/2795 individuals - - - Mohammed Faruq
+/., +?/. 15 2 c.272_273insT r.(?) p.(His92Profs*4), p.(His92ProfsTer4) ACMG likely pathogenic, pathogenic, VUS g.76741492_76741493insA g.76347712_76347713insA BBS10 c.272_273insT, p.C91Lfs*5, c.272 insertion T, c.272>insertion>T, R49W/R49W, 8 more items - BBS10_000094 1 more item PubMed: Chen-2011, PubMed: Deveault-2011, PubMed: Deveault-2012, PubMed: Zacchia 2021 - - Germline, Unknown ? - - - - LOVD
+/., +?/. 13 2 c.273C>G r.(?) p.(Cys91Trp) ACMG likely pathogenic, pathogenic, pathogenic (recessive) g.76741492G>C g.76347712G>C BBS10 c.273C>G, p.C91W, BBS10 c.273C>G, p.Cys91Trp, c.273C>G, c.[1044_1045del];[273C>G], 6 more items - BBS10_000113 heterozygous, heterozygous; unsolved, VKGL data sharing initiative Nederland PubMed: Billingsley-2010, PubMed: Bravo-Gil 2016, PubMed: Deveault-2011, PubMed: Gerth-2008, 4 more items - - CLASSIFICATION record, Germline, Unknown ?, yes - - - - VKGL-NL_Nijmegen
+/., +?/. 2 2 c.285A>T r.(?) p.(Arg95Ser) - likely pathogenic, pathogenic g.76741480T>A g.76347700T>A BBS10 c.285A>T, p.Arg95Ser, c.285A>T - BBS10_000182 heterozygous PubMed: Barrell 2019, PubMed: Redin-2012 - - Germline, In vitro (cloned) ? - - - - LOVD
?/. 1 2 c.285A>Y r.(?) p.(Arg95Ser) - VUS g.76741480T>G - p.R95S - BBS10_000201 - PubMed: Scheidecker 2015 - - Germline - - - - - LOVD
-/., -?/. 2 - c.372T>A r.(?) p.(Ser124=) - benign, likely benign g.76741393A>T g.76347613A>T BBS10(NM_024685.4):c.372T>A (p.S124=) - BBS10_000093 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht, VKGL-NL_AMC
+/., +?/. 3 2 c.378G>A r.(?) p.(Trp126*) ACMG likely pathogenic (recessive), pathogenic, pathogenic (recessive) g.76741387C>T g.76347607C>T c.378G>A, c.378G>A, p.W126*, 1 more item - BBS10_000134 Compound heterozygous PubMed: Liu-2020, PubMed: Tao 2020, PubMed: Wang 2018 - - Germline yes - - - - LOVD
+/. 2 2 c.391del r.(?) p.(Gln131Argfs*4) - pathogenic g.76741374del - c.391delC(h) - BBS10_000198 - PubMed: Janssen-2011 - - Germline - - - - - LOVD
?/. 2 2 c.416A>C r.(?) p.(Gln139Pro) - VUS g.76741349T>G - p.Q139P - BBS10_000200 - PubMed: Scheidecker 2015 - - Germline - - - - - LOVD
+?/., -/., -?/. 7 2 c.424G>A r.(?) p.(Asp142Asn) - benign, likely benign, likely pathogenic g.76741341C>T g.76347561C>T BBS10(NM_024685.4):c.424G>A (p.D142N), c.424G>A, c.42>G>A - BBS10_000071 6 heterozygous, no homozygous; Clinindb (India), VKGL data sharing initiative Nederland PubMed: Duelund Hjortshoj-2010, PubMed: Feuillan-2011, PubMed: Jeziorny-2020, 1 more item - rs142863601 CLASSIFICATION record, Germline yes 0.01, 6/2795 individuals - - - Joshi Stephen, VKGL-NL_Utrecht, VKGL-NL_Nijmegen, Mohammed Faruq
?/. 1 - c.442T>C r.(?) p.(Tyr148His) - VUS g.76741323A>G - BBS10(NM_024685.4):c.442T>C (p.Y148H) - BBS10_000135 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. 1 - c.462G>A r.(?) p.(Leu154=) - benign g.76741303C>T g.76347523C>T BBS10(NM_024685.4):c.462G>A (p.L154=) - BBS10_000088 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+?/. 1 2 c.473C>A r.(?) p.(Ser158*) - likely pathogenic g.76741292G>T - c.473C>A - BBS10_000181 - PubMed: Feuillan-2011 - - Germline - - - - - LOVD
+/. 1 - c.488_489del r.(?) p.(Arg163AsnfsTer4) - pathogenic g.76741281_76741282del g.76347501_76347502del BBS10(NM_024685.4):c.488_489delGA (p.R163Nfs*4) - BBS10_000108 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/., ?/. 2 - c.505T>C r.(?) p.(Ser169Pro) - likely benign, VUS g.76741260A>G g.76347480A>G BBS10(NM_024685.4):c.505T>C (p.S169P) - BBS10_000107 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Nijmegen
+/., +?/. 3 2 c.509T>C r.(?) p.(Leu170Ser) - likely pathogenic, pathogenic g.76741256A>G - c.509T>C, c.509T>C/c.509T>C (p.L170S) - BBS10_000156 - PubMed: Esposito 2017, PubMed: Muller-2010, PubMed: Stoetzel 2006 - - Germline - - - - - LOVD
+/., +?/. 4 2 c.530A>G r.(?) p.(Tyr177Cys) - likely pathogenic, pathogenic, pathogenic (recessive) g.76741235T>C g.76347455T>C 530A/G (Y177C), BBS10 p.Tyr177Cys (c.530A>G) Homozygous, c.[530A>G];[530A>G] - BBS10_000131 Homozygous PubMed: Atmis 2019, PubMed: Harville-2010, PubMed: Mary-2019 - - Germline ? 0/90 ethnically matched controls - - - LOVD
+/. 1 - c.531C>A r.(?) p.(Tyr177Ter) ACMG pathogenic g.76741234G>T g.76347454G>T BBS10 c.531C>A, p.Y177X - BBS10_000211 heterozygous; solved PubMed: Zacchia 2021 - - Unknown ? - - - - LOVD
+/., +?/. 2 2 c.539G>A r.(?) p.(Gly180Glu) ACMG likely pathogenic (recessive), pathogenic g.76741226C>T g.76347446C>T c.539G>A(H), c.539G>A, p.G180E - BBS10_000197 Compound heterozygous PubMed: Janssen-2011, PubMed: Tao 2020 - - Germline yes - - - - LOVD
+/. 4 2 c.559_561del r.(?) p.(His187del) - pathogenic (recessive) g.76741204_76741206del - c.[559_561del];[559_561del] - BBS10_000213 - PubMed: Mary-2019 - - Germline - - - - - LOVD
+/., +?/. 2 2 c.563A>C r.(?) p.(Lys188Thr) - likely pathogenic, pathogenic g.76741202T>G - [K188T;A636V]+[K188T;A636V], [p.M390R];[p.M390R] - BBS10_000180 - PubMed: Billingsley-2010, PubMed: Deveault-2011 - - Germline, Unknown - - - - - LOVD
+?/. 1 - c.578T>C r.(?) p.(Leu193Ser) - likely pathogenic g.76741187A>G g.76347407A>G BBS10, variant 1: c.578T>C/p.L193S, variant 2: c.578T>C/p.L193S - BBS10_000209 possibly solved, homozygous PubMed: Weisschuh 2020 - - Germline yes - - - - LOVD
+/., +?/. 2 - c.590A>G r.(?) p.(Tyr197Cys) - likely pathogenic, pathogenic (recessive) g.76741175T>C g.76347395T>C 12:76741175T>C ENST00000393262.3:c.590A>G (Tyr197Cys), BBS10 c.590A>G, p.Tyr197Cys - BBS10_000117 heterozygous PubMed: Carss 2017, PubMed: Turro 2020 - - Germline, Germline/De novo (untested) ? - - - - LOVD
+?/. 1 2 c.592_597del r.(?) p.(Phe198_Phe199del) - likely pathogenic g.76741168_76741173del - c.591delCTTTTT - BBS10_000155 - PubMed: Muller-2010 - - Germline - - - - - LOVD
+?/. 1 - c.602G>A r.(?) p.(Cys201Tyr) ACMG likely pathogenic (recessive) g.76741163C>T g.76347383C>T c.602G>A, p.C201Y - BBS10_000204 Compound heterozygous PubMed: Tao 2020 - - Germline yes - - - - LOVD
+/., ?/. 3 2 c.641T>A c.641T>A, r.(?) p.(Val214Glu) ACMG pathogenic, VUS g.76741124A>T g.76347344A>T BBS10 c.641T>A, p.(Val214Glu), BBS10 c.641T>A, p.V214E, c.641T>A/c.641T>A (p.V214E) - BBS10_000154 homozygous, homozygous; solved PubMed: Esposito 2017, PubMed: Manara 2019, PubMed: Zacchia 2021 - - Germline, Unknown ? - - - - LOVD
+?/. 1 - c.669_674dup r.(?) p.(Gly224_Val225dup) - likely pathogenic g.76741094_76741099dup g.76347314_76347319dup 665_666insTGTTGG - BBS10_000120 - PubMed: Stone 2017 - - Germline - - - - - LOVD
+?/. 1 2 c.680_681delinsAA r.(?) p.(Gly227Glu) - likely pathogenic g.76741084_76741085delinsTT - c.680_681delGCinsAA - BBS10_000210 - PubMed: Jeziorny-2020 - - Germline - - - - - LOVD
+/. 1 - c.687del r.(?) p.(Val230Phefs*7) - pathogenic g.76741078del g.76347298del BBS10 c.687del, p.(Val230Phefs*7) - BBS10_000224 heterozygous PubMed: Barabino 2020 - - In vitro (cloned) ? - - - - LOVD
+?/. 1 2 c.687delT r.(?) p.(Val230Phefs*7) - VUS g.76741078delA - c.687delT - BBS10_000179 - PubMed: Chen-2011 - - Germline - - - - - LOVD
+?/. 2 2 c.719T>G r.(?) p.(Val240Gly) - likely pathogenic g.76741046A>C - c.719T>G - BBS10_000153 - PubMed: Muller-2010, PubMed: Stoetzel 2006 - - Germline - - - - - LOVD
+/., +?/. 4 2 c.724del r.(?) p.(Gln242Argfs*17) - likely pathogenic, pathogenic (recessive) g.76741041del - c.724delC, c.[724del];[724del] - BBS10_000152 - PubMed: Mary-2019, PubMed: Muller-2010, PubMed: Stoetzel 2006 - - Germline - - - - - LOVD
+/., +?/. 10 2 c.728_731del r.(?) p.(Lys243Ilefs*15), p.(Lys243IlefsTer15) - likely pathogenic, pathogenic (recessive) g.76741034_76741037del, g.76741037_76741040del g.76347257_76347260del BBS10 c.995_999delAAGA, p.Q242fs258X, BBS10 K243IfsX15, c.728_731del p.(K243Ifs*15), 1 more item - BBS10_000126 2 more items PubMed: Abu-Safieh-2012, Stoetzel 2006, PubMed: Fieggen 2016, PubMed: Mary-2019, PubMed: Patel 2016, 1 more item - - Germline yes 0/96 ethnically matched controls, 50/74 BBS families (67%) homozygous, 2 heterozygous - - - LOVD
+?/., -/., -?/., ?/. 7 2 c.765G>A c.765G>A, r.(?) p.(Met255Ile) ACMG benign, likely benign, likely pathogenic, VUS g.76741000C>T g.76347220C>T BBS10 c.765G>A, p.(Met255Ile), BBS10(NM_024685.4):c.765G>A (p.M255I, p.(Met255Ile)), 2 more items - BBS10_000087 heterozygous, VKGL data sharing initiative Nederland PubMed: Duelund Hjortshoj-2010, PubMed: Jespersgaar 2019, PubMed: Manara 2019 - rs139658279 CLASSIFICATION record, Germline ?, yes 0/100 ethnically matched control chromosomes - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Utrecht, VKGL-NL_AMC
+/. 1 - c.766C>T r.(?) p.(Arg256Ter) - pathogenic g.76740999G>A g.76347219G>A BBS10(NM_024685.4):c.766C>T (p.R256*) - BBS10_000086 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-?/. 1 - c.783A>G r.(?) p.(Thr261=) - likely benign g.76740982T>C - BBS10(NM_024685.4):c.783A>G (p.T261=) - BBS10_000216 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 1 2 c.815_822dup r.(?) p.(Phe275Aspfs*6) - pathogenic g.76740944_76740951dup g.76347164_76347171dup F275fsX281 - BBS10_000036 - {PMID:Waters 2015:25564561), Journal: Waters 2015 - - Germline yes - - - - Johan den Dunnen
+?/. 2 2 c.850C>T r.(?) p.(Gln284*) - likely pathogenic, likely pathogenic (recessive) g.76740915G>A - p.(Gln284*) - BBS10_000196 - PubMed: Alvarez-Satta 2017, PubMed: Castro Sanchez 2015 - - Germline yes - - - - Johan den Dunnen
+?/., -?/., ?/. 4 2 c.886G>A r.(?) p.(Ala296Thr) - likely benign, likely pathogenic, VUS g.76740879C>T g.76347099C>T BBS10(NM_024685.4):c.886G>A (p.A296T), [A296T]+[=], [p.D104A];[p.R632P] - BBS10_000085 normal 2nd chromosome, VKGL data sharing initiative Nederland PubMed: Billingsley-2010, PubMed: Deveault-2011 - - CLASSIFICATION record, Germline, Unknown - - - - - VKGL-NL_Rotterdam, VKGL-NL_Utrecht
+/. 1 2 c.899A>C r.(?) p.(His300Pro) - pathogenic g.76740866T>G - c.899A>C(h) - BBS10_000195 1 more item PubMed: Janssen-2011 - - Germline - - - - - LOVD
+?/. 3 2 c.907_910delAGTC r.(?) p.(Ser303Argfs*3) - VUS g.76740855_76740858delGACT - c.907_910delAGTC - BBS10_000178 - PubMed: Chen-2011 - - Germline - - - - - LOVD
+/., +?/. 4 2 c.909_912del r.(?) p.(Ser303Argfs*3), p.(Ser303ArgfsTer3) - likely pathogenic, pathogenic, pathogenic (recessive) g.76740853_76740856del, g.76740855_76740858del g.76347075_76347078del BBS10 c.909_912del, p.(Ser303Argfs*3), c.909delTCAG, S303fsX305 - BBS10_000012 heterozygous PubMed: Barabino 2020, PubMed: Boissel 2017, PubMed: Muller-2010, PubMed: Stoetzel 2006, 1 more item - - Germline, In vitro (cloned) ?, yes - - - - Johan den Dunnen
+/. 1 2 c.926T>C r.(?) p.(Leu309Pro) - pathogenic g.76740839A>G - c.926T>C(p.Leu309Pro);het - BBS10_000194 - PubMed: Lindstrand-2014 - - Germline - - - - - LOVD
+/., +?/. 12 2 c.931T>G r.(?) p.(Ser311Ala) - likely pathogenic, pathogenic, pathogenic (recessive) g.76740834A>C g.76347054A>C BBS10, variant 1: c.931T>G/p.S311A, variant 2: c.931T>G/p.S311A, c.931T>G, S311A, S311A/V11G, 1 more item - BBS10_000091 solved, homozygous, VKGL data sharing initiative Nederland PubMed: Ece Solmaz 2015, PubMed: Laurier-2006, PubMed: Muller-2010, PubMed: Stoetzel 2006, 1 more item - rs137852837 CLASSIFICATION record, Germline yes 0/107 Lebanese controls; 0/96 French controls - - - VKGL-NL_Nijmegen
?/. 1 - c.955G>C r.(?) p.(Val319Leu) - VUS g.76740810C>G g.76347030C>G BBS10(NM_024685.4):c.955G>C (p.V319L) - BBS10_000106 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/. 1 - c.957T>C r.(?) p.(Val319=) - benign g.76740808A>G g.76347028A>G BBS10(NM_024685.4):c.957T>C (p.V319=) - BBS10_000105 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 1 - c.959_962del r.(?) p.(Ser320IlefsTer5) - pathogenic g.76740810_76740813del g.76347030_76347033del BBS10(NM_024685.4):c.959_962delGTTA (p.S320Ifs*5) - BBS10_000084 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/., +?/. 2 2 c.962A>G c.962A>G, r.(?) p.(Tyr321Cys) ACMG likely pathogenic, pathogenic g.76740803T>C g.76347023T>C BBS10 c.962A>G, p.(Tyr321Cys), c.2137_2140del/c.962A>G (p.K713Ffs*16/p.Y321C) - BBS10_000151 heterozygous PubMed: Esposito 2017, PubMed: Manara 2019 - - Germline ? - - - - LOVD
+?/. 1 2 c.963T>G r.(?) p.(Tyr321*) - likely pathogenic g.76740802A>C - c.963T>G - BBS10_000150 - PubMed: Muller-2010, PubMed: Stoetzel 2006 - - Germline - - - - - LOVD
-/., -?/. 2 - c.966T>C r.(?) p.(Tyr322=) - benign, likely benign g.76740799A>G g.76347019A>G BBS10(NM_024685.4):c.966T>C (p.Y322=) - BBS10_000083 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht, VKGL-NL_AMC
+?/., -?/. 2 2 c.968C>T r.(?) p.(Ala323Val) - likely benign, likely pathogenic g.76740797G>A g.76347017G>A BBS10 p.Ala323Val (c.968C>T) Homozygous, c.968C>T - BBS10_000177 Homozygous PubMed: Atmis 2019, PubMed: Chen-2011 - - Germline ? - - - - LOVD
+?/. 1 - c.989T>C r.(?) p.(Val330Ala) - likely pathogenic g.76740776A>G g.76346996A>G BBS10 c.989T>C, p.Val330Ala - BBS10_000220 heterozygous PubMed: Barrell 2019 - - In vitro (cloned) ? - - - - LOVD
+?/. 3 2 c.992T>C r.(?) p.(Val331Ala) - likely pathogenic g.76740773A>G - c.992T>C/p.(Val331Ala)b,c, p.(Val331Ala) - BBS10_000193 - PubMed: Alvarez-Satta-2014, PubMed: Castro Sanchez 2015 - - Germline - - - - - LOVD
+?/., ?/. 2 2 c.1013_1015del r.(?) p.(Glu338del) ACMG likely pathogenic, VUS g.76740750_76740752del, g.76740755_76740757del g.76346975_76346977del c.1009_1011delGAA/p.(Glu337del)b - BBS10_000192 ACMG PM2, PM4, PM1_SUPPORTING PubMed: Alvarez-Satta-2014, PubMed: Weisschuh 2024 - - Germline - 0/100 chromosomes - - - Johan den Dunnen
+/. 1 - c.1024dup r.(?) p.(Ile342AsnfsTer20) - pathogenic (recessive) g.76740741dup g.76346961dup 1024_1025insA - BBS10_000125 - PubMed: Ece Solmaz 2015 - - Germline - - - - - LOVD
+/., +?/. 2 2 c.1044_1045del r.(?) p.(Pro350Ilefs*11) - likely pathogenic, pathogenic (recessive) g.76740720_76740721del - c.1043delTT, c.[1044_1045del];[273C>G] - BBS10_000149 - PubMed: Mary-2019, PubMed: Muller-2010, PubMed: Stoetzel 2006 - - Germline - - - - - LOVD
+?/. 2 - c.1044_1045delTT r.(?) p.(Pro350Ilefs*11) - likely pathogenic g.76740721_76740722del g.76346941_76346942del BBS10 c.1044-1045delTT , p.Pro350IlefsX11, BBS10 c.1044-1045delTT , p.Pro350IlefsX12 - BBS10_000219 heterozygous PubMed: Putoux 2010 - - Germline yes - - - - LOVD
+/. 1 - c.1046T>C r.(?) p.(Leu349Ser) - pathogenic g.94793953T>C g.93781725T>C TMEM67 c.1046T>C, p.(Leu349Ser) - TMEM67_000014 heterozygous PubMed: Barabino 2020 - - In vitro (cloned) ? - - - - LOVD
+/., +?/. 3 2 c.1063C>T r.(?) p.(Gln355*) ACMG likely pathogenic (recessive), pathogenic, pathogenic (recessive) g.76740702G>A g.76346922G>A c.1063C>T, c.1063C>T, p.Q355*, 1 more item - BBS10_000133 Compound heterozygous PubMed: Liu-2020, PubMed: Tao 2020, PubMed: Wang 2018 - - Germline yes - - - - LOVD
+/., +?/. 5 2 c.1091del c.1091del, r.(?) p.(Asn364Thrfs*5), p.(Asn364ThrfsTer5) ACMG likely pathogenic, pathogenic, pathogenic (recessive) g.76740674del, g.76740675del g.76346895del BBS10 c.1091del, p.(Asn364Thrfs*5), c.1091delA, c.[1143T>G];[1091del] - BBS10_000115 heterozygous PubMed: Manara 2019, PubMed: Mary-2019, PubMed: Muller-2010, PubMed: Pierrottet 2014, 1 more item - rs727503818 Germline yes 1/2420 IRD families - - - Global Variome, with Curator vacancy
+?/. 1 2 c.1091delA r.(?) p.(Asn364Thrfs*5) - likely pathogenic g.76740674delT - c.1091delA - BBS10_000176 - PubMed: Schaefer-2011 - - Germline yes - - - - LOVD
+/. 1 2 c.1110G>A r.(?) p.? - pathogenic g.76740655C>T - c.[1110G>A];[1110G>A] - BBS10_000175 - PubMed: Redin-2012 - - Germline yes - - - - LOVD
+/. 1 2 c.1143T>G r.(?) p.(Tyr381*) - pathogenic (recessive) g.76740622A>C - c.[1143T>G];[1091del] - BBS10_000212 - PubMed: Mary-2019 - - Germline - - - - - LOVD
-?/. 2 - c.1158G>A r.(?) p.(Leu386=) - likely benign g.76740607C>T g.76346827C>T BBS10(NM_024685.4):c.1158G>A (p.L386=) - BBS10_000082 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_AMC
+/. 1 2 c.1171_1181dup r.(?) p.(Ser396Leufs*6) - pathogenic g.76740584_76740594dup - c.[1171_1181dupGCATTTATACC] - BBS10_000132 - PubMed: M'hamdi 2014 - - Unknown - - - - - LOVD
+/. 1 2 c.1171_1181dupGCATTTATACC r.(?) p.(Ser396Leufs*6) - pathogenic (recessive) g.76740584_76740594dupGGTATAAATGC - c.[1171_1181dupGCATTTATACC] - BBS10_000174 - PubMed: M'hamdi-2014 - - Germline - - - - - LOVD
?/. 1 - c.1183C>G r.(?) p.(His395Asp) - VUS g.76740582G>C g.76346802G>C - - BBS10_000104 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/. 2 - c.1184A>G r.(?) p.(His395Arg) - likely pathogenic g.76740581T>C g.76346801T>C c.1184A>G , H395R - BBS10_000159 Homozygous PubMed: Chakrabarty 2020 - - Germline yes - - - - LOVD
+?/. 2 2 c.1184dup r.(?) p.(His395Glnfs*14) - likely pathogenic g.76740581dup - c.1184dupA - BBS10_000173 - PubMed: Feuillan-2011 - - Germline - - - - - LOVD
+/. 1 2 c.1189A>G r.(?) p.(Ile397Val) - pathogenic g.76740576T>C g.76346796T>C - - BBS10_000070 no homozygotes in control subjects; variant tested in zebrafish model confirmed deleterious effect PubMed: Lim 2014, Journal: Lim 2014 - rs202042386 Germline yes 3/1791 cases - - - Marianne Vos (LOVD-team)
+?/. 1 2 c.1202G>A r.(?) p.(Gly401Glu) - likely pathogenic g.76740563C>T - c.1660G>A - BBS10_000138 - PubMed: Sathya Priya-2015 - - Germline yes - - - - LOVD
+?/. 1 2 c.1227del r.(?) p.(Gln409Hisfs*22) - likely pathogenic g.76740538del - c.1227delA - BBS10_000137 - PubMed: Sathya Priya-2015 - - Germline yes - - - - LOVD
+?/. 2 2 c.1230T>G r.(?) p.(His410Gln) - likely pathogenic g.76740535A>C - [c.235+1G>A];[c.235+1G>A], [p.F94SfsX9];[p.F94SfsX9] - BBS10_000172 - PubMed: Deveault-2011, PubMed: Deveault-2013 - - Unknown - - - - - LOVD
+/. 1 2 c.1230T>R r.(?) p.(His410Gln) - pathogenic g.76740535A>T - [C91LfsX5]+[H410Q] - BBS10_000171 - PubMed: Billingsley-2010 - - Germline - - - - - LOVD
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