Unique variants in the BBS4 gene

This database is one of the "Eye disease" gene variant databases.
Information The variants shown are described using the NM_033028.4 transcript reference sequence.

130 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 - c.‐2274_24+930d r.0? p.0? ACMG likely pathogenic g.72976295_72979522del g.72683954_72687181del BBS4 c.‐2274_23 + 93 - BBS4_000105 homozygous, error in annotation, should be c.-2274_24+930del, 23 is not an intron boundary PubMed: Dineiro 2020 - - Germline ? - - - - LOVD
-/. 1 - c.-38C>A r.(?) p.(=) - benign g.72978531C>A g.72686190C>A - - BBS4_000027 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-/. 2 - c.-17C>T r.(?) p.(=) - benign g.72978552C>T g.72686211C>T BBS4(NM_033028.4):c.-17C>T - BBS4_000003 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Nijmegen
+/. 1 - c..(76+1_77-1)_(220+1_221-1)del r.? p.(Pro27_Ala74del) ACMG pathogenic (recessive) g.(72987570_73002040)_(73004649_73007631)del g.(72695229_72709699)_(72712308_72715290)del g.73002041_73004648del - BBS4_000030 - PubMed: Hu 2019 - - Germline - - - - - Johan den Dunnen
+/., +?/., ?/. 5 - c.? r.(?), r.0? p.(Glu136Valfs*27), p.0?, p.? ACMG likely pathogenic, pathogenic, VUS g.72978159_72978592del, g.? g.72685818_72686251del c.[(157-?)_(405+?)del];[(157-?)_(405+?)del], M390R/M390R, p.Glu136Valfs*27, [p.L114WfsX28], 1 more item - IGF1R_000000 1 more item PubMed: Deveault-2011, PubMed: Imhoff-2011, PubMed: Knopp 2015, PubMed: Perea-Romero 2021, 1 more item - - Germline, Unknown ?, yes - - - - LOVD
+?/. 1 - c.10G>T r.(?) p.(Glu4Ter) - likely pathogenic g.72978578G>T g.72686237G>T BBS4(NM_033028.5):c.10G>T (p.E4*) - BBS4_000037 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. 1 - c.24+8C>T r.(=) p.(=) - likely benign g.72978600C>T g.72686259C>T BBS4(NM_001252678.1):c.-446+8C>T (p.(=)) - BBS4_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. 1 - c.24+40C>T r.(=) p.(=) - likely pathogenic g.72978632C>T - - - BBS4_000132 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. 1 - c.25-5T>C r.spl? p.? - pathogenic g.72987513T>C g.72695172T>C - - BBS4_000028 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. 1 2 c.31C>T r.(?) p.(Gln11*) ACMG pathogenic g.72987524C>T g.72695183C>T BBS4 c.31C > T, p.Q11* - BBS4_000111 heterozygous PubMed: Meng 2021 - - Germline yes - - - - LOVD
+?/. 1 - c.70A>T r.(?) p.(Lys24*) - likely pathogenic g.72987563A>T g.72695222A>T BBS4 c.70A>T, p.K24X - BBS4_000118 1 more item PubMed: Li 2014 - - Germline yes - - - - LOVD
+/. 2 2i c.76+1G>T r.spl? p.? - pathogenic (recessive) g.72987570G>T - c.[76+1G>T];[657delG] - BBS4_000114 - PubMed: Mary-2019 - - Germline - - - - - LOVD
-/. 3 - c.76+19G>T r.(=) p.(=) - benign g.72987588G>T g.72695247G>T BBS4(NM_033028.4):c.76+19G>T, BBS4(NM_033028.5):c.76+19G>T - BBS4_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Nijmegen
-/. 1 - c.77-5436G>A r.(=) p.(=) - benign g.72996605G>A g.72704264G>A BBS4(NM_033028.4):c.77-5436G>A - BBS4_000006 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-/. 1 - c.77-5226A>G r.(=) p.(=) - benign g.72996815A>G g.72704474A>G BBS4(NM_033028.4):c.77-5226A>G - BBS4_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+/. 1 - c.77-1476_406-309del r.(?) p.(Ala26Glyfs*5) ACMG pathogenic g.73000565_73014826del - - - BBS4_000058 - PubMed: Sharon 2019 - - Germline - 2/2420 IRD families - - - Global Variome, with Curator vacancy
+/. 1 - c.77-1454_221-786del r.(?) p.(Pro27_Ala74del) - pathogenic g.73000587_73006846del - del exon3_4 73000565-73006826del - BBS4_000066 6,261 bp deletion, AluSc8 - AluSx PubMed: Lindstrand 2016 - - Germline - - - - - LOVD
-/., -?/. 3 - c.77-6G>A r.(=) p.(=) - benign, likely benign g.73002035G>A g.72709694G>A BBS4(NM_033028.4):c.77-6G>A, BBS4(NM_033028.5):c.77-6G>A - BBS4_000008 expression cloning mini-gene splicing assay shows no effect on splicing, 1 more item PubMed: Alvarez-Satta 2017 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_Rotterdam, VKGL-NL_Groningen
+/. 7 10, 2i_4i c.(76+1_77-1)_(220+1_221-1)del r.(?), r.? p.? - pathogenic g.(72987570_73002040)_(73004649_73007631)del g.(72695229_72709699)_(72712308_72715290)del BBS4 exon 3 and 4 6 kb deletion, ex 3-4del - BBS4_000030 homozygous Sharon, submitted, PubMed: Mykytyn 2001 - - Germline yes - - - - Dror Sharon
+?/. 3 3 c.77_156dup r.(?) p.(Ala53Leufs*13) - likely pathogenic g.73002041_73002120dup - exon3dup - BBS4_000094 - PubMed: Feuillan-2011 - - Germline - - - - - LOVD
+/. 1 3_4 c.77_220del r.(?) p.(Pro27_Ala74del) - pathogenic g.73002041_73004648del - c.77_220del - BBS4_000102 - PubMed: Fattahi 2014 - - Germline - - - - - LOVD
+?/. 1 - c.129T>G r.(?) p.(Tyr43*) - likely pathogenic g.73002093T>G g.72709752T>G BBS4, variant 1: c.129T>G/p.Y43*, variant 2: c.883C>T/p.R295* - BBS4_000108 solved, compound heterozygous PubMed: Weisschuh 2020 - - Germline yes - - - - LOVD
+?/., -?/., ?/. 8 3 c.137A>G r.(?) p.(Lys46Arg) - likely benign, likely benign (recessive), likely pathogenic, VUS g.73002101A>G g.72709760A>G BBS4(NM_033028.4):c.137A>G (p.K46R), BBS4(NM_033028.5):c.137A>G (p.K46R), c.137A>G, [p.N90S], 1 more item - BBS4_000009 conflicting interpretations of pathogenicity; 8 heterozygous, no homozygous; Clinindb (India), 2 more items PubMed: Anasagasti-2013, PubMed: Deveault-2011, PubMed: Lindstrand 2016, 2 more items - rs75295839 CLASSIFICATION record, Germline, Unknown yes 8/2791 individuals, <0.01 - - - VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Utrecht, Mohammed Faruq
+?/., -/. 2 3 c.147A>G r.(=) p.(=) - benign, likely pathogenic g.73002111A>G - 147A>G, K46R - BBS4_000082 - PubMed: Eichers-2009, Fauser 2003, PubMed: Fauser-2003, Mykytyn 2003 - - Germline, Unknown - - - - - LOVD
-?/. 1 - c.157-19A>G r.(=) p.(=) - likely benign g.73004566A>G g.72712225A>G BBS4(NM_033028.4):c.157-19A>G - BBS4_000010 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/., +?/. 7 3i c.157-3C>G r.spl, r.spl? p.(?), p.? - likely pathogenic, pathogenic, pathogenic (recessive) g.73004582C>G g.72712241C>G BBS4, variant 1: c.157-3C>G/p.?, variant 2: c.157-3C>G/p.?, c.[157-3C>G];[157-3C>G], 3 more items - BBS4_000056 solved, homozygous PubMed: Deveault-2011, PubMed: Harville-2010, PubMed: Makrythanasis 2014, PubMed: Mary-2019, 1 more item - - Germline, Unknown ? 0/90 ethnically matched controls - - - Johan den Dunnen
+/., +?/., ?/. 15 3i c.157-2A>G r.157_220del, r.spl, r.spl? p.? ACMG likely pathogenic, pathogenic, VUS g.73004583A>G g.72712242A>G BBS4 IVS3-2A>G, BBS4(NM_033028.5):c.157-2A>G, c.157-2A>G, c.IVS3-2A>G(H), IVS3-2a/g, 1 more item - BBS4_000002 no nucleotide annotation, extrapolated from protein, sequence and databases; homozygous, 1 more item PubMed: Abu-Safieh-2012, Abu-Safieh 2010, PubMed: Abu-Safieh-2013, PubMed: Harville-2010, 4 more items - - CLASSIFICATION record, Germline yes 0.017, 0/108 ethnically matched control chromosomes, 0/90 ethnically matched controls, 1 more item - - - Daniel Trujillano, VKGL-NL_VUmc
+/. 4 3i_4i c.157-1_220+1del r.spl? p.? - pathogenic (recessive) g.73004584_73004649del - c.[157-?_220+?del];[157-?_220+?del] - BBS4_000115 - PubMed: Mary-2019 - - Germline - - - - - LOVD
+/. 1 3i_6i c.(?_157-1)_(405+1)del r.spl? p.? - pathogenic g.73004584_73009192del - Del exon 4+5+6 - BBS4_000073 - PubMed: Muller-2010 - - Germline - - - - - LOVD
+/. 1 3i_6i c.(?_157-1)_(405+1_?)del r.spl? p.? - pathogenic g.73004584_73009192del - Deletion Ex4-5-6 - BBS4_000073 - PubMed: M'hamdi 2014 - - Unknown - - - - - LOVD
+/., +?/. 2 3i_6i c.157-1_405+1del r.spl? p.? - likely pathogenic, pathogenic (recessive) g.73004584_73009192del - deletionexons4,5,6, DelEx4-5-6 - BBS4_000073 - PubMed: Feuillan-2011, PubMed: M'hamdi-2014 - - Germline - - - - - LOVD
-?/. 2 - c.180A>G r.(?) p.(Gln60=) - likely benign g.73004608A>G g.72712267A>G BBS4(NM_033028.4):c.180A>G (p.Q60=) - BBS4_000011 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Utrecht
+?/., ?/. 4 4 c.181G>A r.(?) p.(Glu61Lys) - likely pathogenic, VUS g.73004609G>A - (c.1115_1116delTT; p.Phe372X), [E61K]+[=], [p.Q293E];[p.F372XfsX1] - BBS4_000095 normal 2nd chromosome PubMed: Billingsley-2010, PubMed: Deveault-2011 - - Germline, Unknown - - - - - LOVD
+?/. 1 4 c.218A>G r.(?) p.(Gln73Arg) - likely pathogenic g.73004646A>G - c.218A>G(h) - BBS4_000101 unknown variant 2nd chromosome PubMed: Janssen-2011 - - Germline - 2.3% ; absent in 96 controls - - - LOVD
+/. 1 10 c.220+1G>C r.spl p.? - pathogenic g.73004649G>C g.72712308G>C BBS4 c.220+1G>C - BBS4_000119 homozygous PubMed: Mykytyn 2001 - - Germline yes - - - - LOVD
+/. 1 - c.221-1062_406-847del r.(?) p.(Ala74Glyfs*5) - pathogenic g.73006570_73014288del - del exon5_6 73006570-73014289del - BBS4_000065 7,719 bp deletion, 1 bp microhomology PubMed: Lindstrand 2016 - - Germline - - - - - LOVD
+/. 1 - c.221-1035_405+2929del r.(?) p.(Ala74Glyfs*5) - pathogenic g.73006597_73012120del - del exon5_6 73006584-73012788del - BBS4_000067 6,204 bp deletion, AluSx - AluSg PubMed: Lindstrand 2016 - - Germline - - - - - LOVD
?/. 1 4i c.221-37G>A r.(=) p.(=) - VUS g.73007595G>A - c.221-37G>A - BBS4_000096 - PubMed: Abu-Safieh-2012, Abu-Safieh 2010 - - Germline - - - - - LOVD
-?/. 1 - c.223T>C r.(?) p.(Leu75=) - likely benign g.73007634T>C g.72715293T>C BBS4(NM_001252678.1):c.-299T>C (p.(=)) - BBS4_000039 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. 3 - c.253G>C r.(?) p.(Glu85Gln) - likely pathogenic g.73007664G>C g.72715323G>C BBS4 c.253G>C;p.E85Q - BBS4_000120 homozygous PubMed: Wang 2011 - - Germline yes 0/200 normal matching controls, including 96 from Saudi Arabia - - - LOVD
+?/. 1 - c.262del r.(?) p.(Glu88AsnfsTer54) - likely pathogenic g.73007673del g.72715332del - - BBS4_000070 - PubMed: Patel 2016 - - Germline - - - - - LOVD
-?/. 1 - c.275C>T r.(?) p.(Thr92Ile) - likely benign g.73007686C>T g.72715345C>T BBS4(NM_033028.4):c.275C>T (p.T92I) - BBS4_000040 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 2 - c.289A>C r.(?) p.(Ser97Arg) - VUS g.73007700A>C g.72715359A>C BBS4(NM_001252678.1):c.-233A>C (p.(=)), BBS4(NM_033028.5):c.289A>C (p.S97R) - BBS4_000013 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_AMC
+?/. 1 - c.322G>A r.(?) p.(Ala108Thr) - likely pathogenic g.73007733G>A g.72715392G>A BBS4, variant 1: c.322G>A/p.A108T, variant 2: c.514dup/p.I172Nfs*18 - BBS4_000109 possibly solved, compound heterozygous PubMed: Weisschuh 2020 - - Unknown ? - - - - LOVD
-?/. 1 - c.329C>G r.(?) p.(Ser110Cys) - likely benign g.73007740C>G g.72715399C>G BBS4(NM_001252678.1):c.-193C>G (p.(=)) - BBS4_000041 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/., +?/., ?/. 7 5i c.332+2_332+3insTT c.332+2_332+3insTT, r.spl? p.(?), p.? ACMG likely pathogenic, pathogenic, VUS g.73007745_73007746insTT g.72715404_72715405insTT BBS4 c.332+1G>GTT, spl, BBS4 c.332+1G>GTT, Spl?, BBS4 c.332+2_332+3insTT,, c.332+2_332+3insTT - BBS4_000083 error in annotation, should be c.332+2_332+3insTT, homozygous, no ACMG classification; solved, 2 more items PubMed: Knopp 2015, PubMed: Manara 2019, PubMed: Zacchia 2021 - rs753360929 Germline, Unknown ?, yes - - - - LOVD
-?/. 1 - c.332+8T>C r.(=) p.(=) - likely benign g.73007751T>C g.72715410T>C BBS4(NM_033028.4):c.332+8T>C - BBS4_000014 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+/., -/. 2 5i c.332+27_332+28insA r.(=) p.(=) - benign, pathogenic g.73007770_73007771insA, g.? - c.332+27_28insA - BBS4_000081 - PubMed: Duelund Hjortshoj-2010, PubMed: Hoskins-2003 - - Germline, Unknown - 0.01 - - - LOVD
+?/. 1 - c.333-2A>C r.spl p.? ACMG likely pathogenic g.73009117A>C g.72716776A>C BBS4 c.333-2A>C, p.? - BBS4_000127 heterozygous, probably non-causal incidental finding PubMed: Zhu 2022 - - Germline/De novo (untested) ? - - - - LOVD
+/. 1 - c.336_341del r.(?) p.(Phe112_Leu113del) ACMG pathogenic g.73009122_73009127del g.72716781_72716786del BBS4 c.334_338del, p.112_113del - BBS4_000113 1 more item PubMed: Zacchia 2021 - - Unknown ? - - - - LOVD
+?/. 1 6 c.341del r.(?) p.(Leu114Trpfs*28) - likely pathogenic g.73009127del - p.[L114WfsX28]+[=] - BBS4_000084 - PubMed: Bin-2009 - - Germline yes - - - - LOVD
+/. 1 6 c.341_342insA r.(?) p.(His117Thrfs*2) - pathogenic g.73009127_73009128insA - c.341insA - BBS4_000085 - PubMed: Muller-2010 - - Germline - - - - - LOVD
-/., -?/. 2 - c.372T>C r.(?) p.(Tyr124=) - benign, likely benign g.73009158T>C g.72716817T>C BBS4(NM_033028.4):c.372T>C (p.Y124=), BBS4(NM_033028.5):c.372T>C (p.Y124=) - BBS4_000015 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_AMC
-?/. 1 - c.402T>C r.(?) p.(Asp134=) - likely benign g.73009188T>C - BBS4(NM_033028.5):c.402T>C (p.D134=) - BBS4_000128 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 1 - c.405+1G>A r.spl? p.? - pathogenic g.73009192G>A g.72716851G>A - - BBS4_000055 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-/., ?/. 5 6i c.405+17C>T r.(=), r.(?) p.(=) - benign, VUS g.73009208C>T g.72716867C>T BBS4(NM_033028.4):c.405+17C>T, c.405+17C>T, c.[405+17C>T(;)1248+65C>T] - BBS4_000016 3 homozygous; Clinindb (India), VKGL data sharing initiative Nederland PubMed: Abu-Safieh-2012, Abu-Safieh 2010, PubMed: Duelund Hjortshoj-2010, PubMed: Smaoui 2006, 1 more item - rs80334351 CLASSIFICATION record, Germline - 0.01, 1/19 families BBS, 3/2794 individuals - - - Johan den Dunnen, VKGL-NL_Rotterdam, Mohammed Faruq
+/. 1 10 c.406-2A>C r.spl p.? - pathogenic g.73015133A>C g.72722792A>C BBS4 c.406-2A>C - BBS4_000121 homozygous PubMed: Mykytyn 2001 - - Germline yes - - - - LOVD
+/. 1 - c.406-2A>G r.(?) p.? - pathogenic (recessive) g.73015133A>G g.72722792A>G - - BBS4_000071 - PubMed: Ece Solmaz 2015 - - Germline - - - - - LOVD
+?/. 1 6i_8i c.(405+1_406-1)_(587+1_588-1)del r.? p.? - likely pathogenic g.(73009192_73015134)_(73016997_73020280)del - chr15:73015130–73017001 - BBS4_000106 - PubMed: Ellingsford 2018 - - Germline - - - - - LOVD
+/. 1 6i_8i c.(?_406-1)_(587+1_?)del r.spl? p.? - pathogenic g.73015134_73016997del - Del exon 7+8 - BBS4_000086 - PubMed: Muller-2010 - - Germline - - - - - LOVD
+?/. 1 - c.424G>A r.(?) p.(Gly142Arg) ACMG likely pathogenic g.73015153G>A - - - BBS4_000059 - PubMed: Sharon 2019 - - Germline - 1/2420 IRD families - - - Global Variome, with Curator vacancy
?/. 1 - c.439T>A r.(?) p.(Tyr147Asn) - VUS g.73015168T>A g.72722827T>A - - BBS4_000068 - PubMed: Lindstrand 2016 - - Germline - - - - - LOVD
?/. 1 - c.443T>A r.(?) p.(Leu148Gln) - VUS g.73015172T>A g.72722831T>A BBS4(NM_033028.5):c.443T>A (p.L148Q) - BBS4_000042 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. 1 - c.458A>G r.(?) p.(Lys153Arg) - VUS g.73015187A>G g.72722846A>G BBS4(NM_033028.5):c.458A>G (p.K153R) - BBS4_000017 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/. 1 - c.459+14G>C r.(=) p.(=) - benign g.73015202G>C g.72722861G>C BBS4(NM_033028.4):c.459+14G>C - BBS4_000018 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.493A>C r.(?) p.(Asn165His) - likely benign g.73016902A>C g.72724561A>C BBS4 N165H - BBS4_000122 1 more item PubMed: Katsanis 2002 - - Germline no 0/384 ethnically matched control chromosomes and 96 random control chromosomes - - - LOVD
?/. 1 - c.514A>G r.(?) p.(Ile172Val) - VUS g.73016923A>G - BBS4(NM_033028.4):c.514A>G (p.I172V) - BBS4_000075 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 2 - c.514dup r.(?) p.(Ile172Asnfs*18) - likely pathogenic, likely pathogenic (recessive) g.73016923dup g.72724582dup 513_514insA, BBS4, variant 1: c.322G>A/p.A108T, variant 2: c.514dup/p.I172Nfs*18 - BBS4_000062 possibly solved, compound heterozygous PubMed: Bryant 2018, PubMed: Weisschuh 2020 - rs779047261 Germline, Unknown ? - - - - LOVD
+?/. 1 10 c.584_585dup r.(?) p.(Glu196Trpfs*14) - likely pathogenic g.73016993_73016994dup g.72724652_72724653dup BBS4 c.585-586insTG, premature termination at codon 209 - BBS4_000123 heterozygous; no second allele found PubMed: Mykytyn 2001 - - Unknown ? - - - - LOVD
-?/. 1 - c.587+7G>A r.(=) p.(=) - likely benign g.73017003G>A - BBS4(NM_001252678.1):c.71+7G>A (p.(=)) - BBS4_000117 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.588-9A>G r.(=) p.(=) - likely benign g.73020272A>G g.72727931A>G BBS4(NM_033028.4):c.588-9A>G - BBS4_000019 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 2 10 c.657del r.(?) p.(Lys220Argfs*10) - pathogenic (recessive) g.73021971del - c.[76+1G>T];[657delG] - BBS4_000116 - PubMed: Mary-2019 - - Germline - - - - - LOVD
?/. 1 - c.697C>G r.(?) p.(Pro233Ala) - VUS g.73022011C>G - BBS4(NM_033028.5):c.697C>G (p.(Pro233Ala)) - BBS4_000133 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.712-1G>A r.spl p.? - VUS g.73023645G>A g.72731304G>A - - BBS4_000069 - PubMed: Ellingford 2016 - - Germline - - - - - LOVD
+/. 1 11 c.739C>T r.(?) p.(Gln247*) - pathogenic g.73023673C>T - c.739C>T - BBS4_000087 - PubMed: Muller-2010 - - Germline - - - - - LOVD
-/., -?/. 2 - c.748G>A r.(?) p.(Gly250Arg) - benign, likely benign g.73023682G>A g.72731341G>A BBS4(NM_033028.4):c.748G>A (p.G250R), BBS4(NM_033028.5):c.748G>A (p.G250R) - BBS4_000021 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht, VKGL-NL_AMC
+/. 3 - c.777_778del r.(?) p.(Tyr259Ter) - pathogenic g.73023711_73023712del g.72731370_72731371del BBS4(NM_033028.4):c.777_778delCA (p.Y259*), BBS4(NM_033028.5):c.777_778delCA (p.Y259*) - BBS4_000022 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht, VKGL-NL_Nijmegen, VKGL-NL_VUmc
+/. 1 11 c.829G>A r.(?) p.(Gly277Arg) - pathogenic g.73023763G>A - c.829G>A - BBS4_000103 - PubMed: Fattahi 2014 - - Germline - - - - - LOVD
?/. 1 - c.863C>T r.(?) p.(Ala288Val) - VUS g.73023797C>T g.72731456C>T BBS4(NM_033028.5):c.863C>T (p.A288V) - BBS4_000043 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+?/. 1 - c.883C>G r.(?) p.(Arg295Gly) - likely pathogenic (recessive) g.73023914C>G g.72731573C>G - - BBS4_000063 - PubMed: Bryant 2018 - - Germline - - - - - LOVD
+/., +?/. 4 - c.883C>T r.(?) p.(Arg295*), p.(Arg295Ter) - likely pathogenic, pathogenic g.73023914C>T g.72731573C>T BBS4(NM_033028.4):c.883C>T (p.R295*), 2 more items - BBS4_000057 possibly solved, compound heterozygous, solved, compound heterozygous, 1 more item PubMed: Weisschuh 2020 - - CLASSIFICATION record, Germline, Unknown ?, yes - - - - VKGL-NL_Utrecht, VKGL-NL_Nijmegen
+/., +?/. 3 10 c.884G>C r.(?) p.(Arg295Pro) ACMG likely pathogenic, pathogenic g.73023915G>C g.72731574G>C BBS4 c.884G>C, p.(Arg295Pro), BBS4 R295P - BBS4_000060 homozygous, homozygous; no nucleotide annotation, extrapolated from protein and databases PubMed: Heon 2005, PubMed: Mykytyn 2001, PubMed: Sharon 2019 - - Germline yes 2/2420 IRD families - - - Global Variome, with Curator vacancy
-/. 2 - c.906= r.(=) p.(Phe302=) - benign g.73023937T>C g.72731596T>C BBS4(NM_033028.4):c.906T>C (p.F302=), BBS4(NM_033028.5):c.906T>C (p.F302=) - BBS4_000023 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Groningen
+/. 1 12 c.927T>G r.(?) p.(Asn309Lys) - pathogenic g.73023958T>G - c.927T>G - BBS4_000088 - PubMed: Muller-2010 - - Germline - - - - - LOVD
+/. 1 12 c.932G>A r.(?) p.(Gly311Asp) ACMG pathogenic g.73023963G>A g.72731622G>A BBS4 c.932G > A, p.G311D - BBS4_000112 heterozygous PubMed: Meng 2021 - - Germline yes - - - - LOVD
-?/. 1 - c.980T>C r.(?) p.(Leu327Pro) - likely benign g.73024011T>C g.72731670T>C BBS4 L327P - BBS4_000124 1 more item PubMed: Katsanis 2002 - - Germline no 0/384 ethnically matched control chromosomes and 96 random control chromosomes - - - LOVD
-?/. 1 - c.987G>A r.(?) p.(Ala329=) - likely benign g.73024018G>A g.72731677G>A BBS4(NM_033028.4):c.987G>A (p.A329=) - BBS4_000044 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. 1 - c.1049A>G r.(?) p.(Asn350Ser) - benign g.73027466A>G g.72735125A>G BBS4(NM_033028.4):c.1049A>G (p.N350S) - BBS4_000045 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+/. 1 13 c.1052T>G r.(?) p.(Leu351Arg) - pathogenic g.73027469T>G - c.1052T>G - BBS4_000076 - PubMed: Hichri-2005 - - Unknown - - - - - LOVD
+/. 1 13 c.1061C>T r.(?) p.? - pathogenic g.73027478C>T - c.1061C>T - BBS4_000089 C not found at position 1110, found T instead. PubMed: Hoskins-2003 - - Germline - - - - - LOVD
-/. 7 13 c.1061T>C r.(?) p.(Ile354Thr) - benign, benign (recessive) g.73027478T>C g.72735137T>C BBS4(NM_033028.4):c.1061T>C (p.I354T), BBS4(NM_033028.5):c.1061T>C (p.I354T), p.Ile354Thr - BBS4_000024 VKGL data sharing initiative Nederland PubMed: Anasagasti-2013, PubMed: Smaoui 2006 - rs2277598 CLASSIFICATION record, Germline yes 0.45, 10/19 families BBS - - - Johan den Dunnen, VKGL-NL_Rotterdam, VKGL-NL_Groningen
+?/. 1 13_16 c.(?_1077-1)_(1601+1_?)del r.spl? p.? - likely pathogenic g.73027493_73029970del - [c.1077-?_1601+?] - BBS4_000077 - PubMed: Sathya Priya-2015 - - Germline yes - - - - LOVD
+/. 1 - c.1083C>A r.(?) p.(Tyr361Ter) - pathogenic g.73027500C>A g.72735159C>A - - BBS4_000029 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/., +?/., ?/. 9 13 c.1091C>A c.1091C>A, r.(?) p.(Ala364Glu) ACMG likely pathogenic, likely pathogenic (recessive), pathogenic, VUS g.73027508C>A g.72735167C>A A364E, BBS4 c.1091C>A, A364E, BBS4 c.1091C>A, p.(Ala364Glu), BBS4 c.1091C>A, p.A364E, 3 more items - BBS4_000025 four mutant alleles are potentially necessary for disease pathogenesis., heterozygous, homozygous, 3 more items PubMed: Eichers-2009, Badano 2003, PubMed: Hoskins-2003, PubMed: Katsanis 2002, PubMed: Manara 2019, 3 more items - rs28938468 CLASSIFICATION record, Germline, Unknown ?, yes 0/384 control chromosomes (including 84 Kurdish chromosomes) - - - VKGL-NL_Rotterdam, VKGL-NL_Nijmegen
+?/. 2 13 c.1103A>G r.(?) p.(Asp368Gly) - likely pathogenic g.73027520A>G g.72735179A>G BBS4, variant 1: c.1103A>G/p.D368G, variant 2: c.1103A>G/p.D368G, c.1103A>G - BBS4_000097 solved, homozygous PubMed: Feuillan-2011, PubMed: Weisschuh 2020 - - Germline, Unknown ? - - - - LOVD
+/. 1 - c.1106+2T>A r.(?) p.(?) ACMG pathogenic g.73027525T>A g.72735184T>A BBS4 c.1106+2T>A, p.(?), c.1106+2T>A, p.(?), ABCA4 c.5882G>A, p.(Gly1961Glu) - BBS4_000074 homozygous PubMed: Jespersgaar 2019 - - Germline ? - - - - LOVD
-/. 1 - c.1106+20T>C r.(=) p.(=) - benign g.73027543T>C g.72735202T>C BBS4(NM_033028.4):c.1106+20T>C - BBS4_000046 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 13i c.1107-45T>C r.(=) p.(=) - VUS g.73028121T>C - c.1107-45T>C - BBS4_000098 - PubMed: Abu-Safieh-2012, Abu-Safieh 2010 - - Germline - - - - - LOVD
+?/. 1 - c.1107-10_1107-7del r.spl? p.(?) - likely pathogenic g.73028156_73028159del g.72735815_72735818del BBS4, variant 1: c.883C>T/p.R295*, variant 2: c.1107-10_1107-7del/p.? - BBS4_000110 possibly solved, compound heterozygous PubMed: Weisschuh 2020 - - Unknown ? - - - - LOVD
-?/. 1 - c.1107-7G>A r.(=) p.(=) - likely benign g.73028159G>A - BBS4(NM_033028.4):c.1107-7G>A - BBS4_000126 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
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