Unique variants in the RGR gene

This database is one of the "Eye disease" gene variant databases.
Information The variants shown are described using the NM_002921.3 transcript reference sequence.

46 entries on 1 page. Showing entries 1 - 46.
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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.-111A>G r.(=) p.(=) - likely benign g.86004736A>G - c.-111A>G - RGR_000031 - PubMed: Ksantini-2010 - - Germline - 72.0% of 216 patients - - - LOVD
?/. 1 - c.5C>T r.(?) p.(Ala2Val) - VUS g.86004851C>T g.84245095C>T - - RGR_000012 - PubMed: Koyanagi 2019, Journal: Koyanagi 2019 - - Germline - 1/1204 cases with retinitis pigmentosa - - - Yoshito Koyanagi
-/. 2 1 c.19C>T r.(=), r.(?) p.(=), p.(Leu7=) - benign g.86004865C>T g.84245109C>T c.19C>T - RGR_000010 VKGL data sharing initiative Nederland PubMed: Ksantini-2010 - rs11200938 CLASSIFICATION record, Germline - 72.0% of 216 patients - - - VKGL-NL_Nijmegen
-/. 3 1 c.27T>C r.(=), r.(?) p.(=), p.(Thr9=) - benign g.86004873T>C g.84245117T>C c.27T>C, RGR(NM_002921.4):c.27T>C (p.T9=) - RGR_000003 VKGL data sharing initiative Nederland PubMed: Ksantini-2010 - rs2279227 CLASSIFICATION record, Germline - 72.0% of 216 patients - - - VKGL-NL_Groningen, VKGL-NL_Nijmegen
?/. 1 - c.34G>A r.(?) p.(Gly12Arg) - VUS g.86004880G>A g.84245124G>A RGR(NM_002921.3):c.34G>A (p.G12R) - RGR_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 1i c.79+59C>T r.(=) p.(=) - likely benign g.86004984C>T - c.79+59C>T - RGR_000032 - PubMed: Ksantini-2010 - - Germline - 2.0% of 216 patients - - - LOVD
?/. 1 - c.88G>A r.(?) p.(Gly30Ser) - VUS g.86007355G>A - RGR(NM_002921.3):c.88G>A (p.G30S) - RGR_000037 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.110C>T r.(?) p.(Thr37Ile) - VUS g.86007377C>T g.84247621C>T - - RGR_000013 - PubMed: Koyanagi 2019, Journal: Koyanagi 2019 - rs774849552 Germline - 1/1204 cases with retinitis pigmentosa - - - Yoshito Koyanagi
?/. 1 2 c.123C>T r.(=) p.(=) - VUS g.86007390C>T - - - RGR_000029 1 more item PubMed: Singh 2009 - - Germline - - - - - LOVD
-?/. 2 - c.183G>A r.(?) p.(Ala61=) - likely benign g.86007450G>A g.84247694G>A RGR(NM_002921.3):c.183G>A (p.A61=), RGR(NM_002921.4):c.183G>A (p.A61=) - RGR_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_AMC
+?/., ?/. 17 2 c.196A>C r.(?) p.(Ser66Arg) ACMG likely pathogenic, likely pathogenic (recessive), VUS, VUS (!) g.86007463A>C g.84247707A>C c.196A>C, NM_001012720, c.196A>C, p.Ser66Arg, RGR c.196A>C, (p.Ser66Arg), RGR Ser66Arg, 1 more item - RGR_000011 ACMG PM2, heterozygous, Reevaluation of the previously reported causative status of this variant, 2 more items PubMed: Arno-2016, PubMed: Collin-2011, PubMed: Ezquerra-Inchausti 2018, PubMed: Mermeklieva 2021, 3 more items - - CLASSIFICATION record, Germline, Unknown ?, yes 0/95 unaffected controls - - - Johan den Dunnen, VKGL-NL_Nijmegen
?/. 1 - c.229C>T r.(?) p.(Leu77Phe) - VUS g.86007496C>T - RGR(NM_002921.3):c.229C>T (p.L77F) - RGR_000044 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 2 2 c.236G>A r.(?) p.(Arg79His) - likely pathogenic g.86007503G>A - c.236G>A - RGR_000042 - PubMed: Maggi_2021 - - Germline - - - - - LOVD
?/. 1 2i_3i c.(236+1_237-1)_(370+1_371-1)del r.? p.? - VUS g.(86007504_86008665)_(86008800_86012612)del - chr10:86008662–86008804 - RGR_000041 - PubMed: Ellingsford 2018 - - Germline - - - - - LOVD
-?/., ?/. 3 - c.262G>A r.(?) p.(?), p.(Gly88Ser) - likely benign, VUS g.86008691G>A g.84248935G>A NM_001012720.1:c.250G>A, 1 more item - RGR_000017 VKGL data sharing initiative Nederland PubMed: Wang 2014 - rs116754489 CLASSIFICATION record, Germline - - - - - VKGL-NL_Rotterdam, VKGL-NL_AMC
+/., +?/. 4 - c.266C>A r.(?) p.(Ser89*), p.(Ser89Ter) - pathogenic, VUS (!) g.86008695C>A g.84248939C>A RGR c.[=];[=], RGR c.266C>A (p.S89*) - RGR_000025 affected person without the mutation, not in 624 control chromosomes, 1 more item PubMed: Li 2016, PubMed: Sun 2015 - - Germline no 1/596 chromosomes - - - LOVD
?/. 1 - c.269A>G r.(?) p.(Asp90Gly) - VUS g.86008698A>G g.84248942A>G - - RGR_000026 - PubMed: Xu 2014 - - Germline - 1/314 case chromosomes - - - LOVD
?/. 2 3 c.286G>A r.(?) p.(Gly96Ser) ACMG VUS g.86008715G>A g.84248959G>A NM_001012720G274A - RGR_000028 ACMG PM2 PubMed: Katagiri 2014, PubMed: Weisschuh 2024 - - Germline - - - - - Johan den Dunnen
-/., -?/. 2 - c.330T>C r.(?) p.(Ser110=) - benign, likely benign g.86008759T>C g.84249003T>C RGR(NM_002921.3):c.330T>C (p.S110=), RGR(NM_002921.4):c.330T>C (p.S110=) - RGR_000006 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_AMC
?/. 1 - c.349C>T r.(?) p.(Arg117Cys) ACMG VUS g.86008778C>T g.84249022C>T - - RGR_000046 ACMG PM2; no variant 2nd chromosome PubMed: Weisschuh 2024 - - Germline/De novo (untested) - - - - - Johan den Dunnen
?/. 1 - c.350G>A r.(?) p.(Arg117His) - VUS g.86008779G>A g.84249023G>A - - RGR_000022 - PubMed: Riazuddin 2017 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.371-15C>A r.(=) p.(=) - likely benign g.86012598C>A g.84252842C>A RGR(NM_002921.4):c.371-15C>A - RGR_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/., +?/. 2 4 c.371G>A r.(?) p.(Arg124His) ACMG likely pathogenic, pathogenic g.86012613G>A g.84252857G>A RGR NM_001012720: g.3980G>A, c.359G>A, p.R120H - RGR_000002 different transcript: NM_001012722.1(RGR):c.359G>A PubMed: Xu 2020 - - Germline, Unknown yes - - - - Feng Wang
?/. 1 - c.397G>A r.(?) p.(Val133Ile) ACMG VUS g.86012639G>A g.84252883G>A RGR:NM_002921 c.G397A, p.V133I - RGR_000040 heterozygous, individual unsolved, causality of variants unknown PubMed: Rodriguez-Munoz 2020 - - Germline ? - - - - LOVD
?/. 1 - c.404T>C r.(?) p.(Leu135Pro) - VUS g.86012646T>C g.84252890T>C - - RGR_000014 - PubMed: Koyanagi 2019, Journal: Koyanagi 2019 - - Germline - 1/1204 cases with retinitis pigmentosa - - - Yoshito Koyanagi
+?/. 1 4 c.454C>A r.(?) p.(Leu152Met) - likely pathogenic g.86012696C>A - c.454C>A - RGR_000043 - PubMed: Booij-2011 - - Germline - - - - - LOVD
+?/., -/., -?/. 5 4 c.466C>A r.(?) p.(His156Asn) - benign (dominant), benign (recessive), likely benign, likely pathogenic g.86012708C>A g.84252952C>A c.466C>A, NM_001012720.1:c.454C>A, RGR(NM_002921.3):c.466C>A (p.H156N) - RGR_000021 VKGL data sharing initiative Nederland PubMed: Eisenberger-2013, PubMed: Holtan 2020, PubMed: Ksantini-2010 - rs150808273 CLASSIFICATION record, Germline - 0.5% of 216 patients; 0/100 controls, 1/899 cases - - - Global Variome, with Curator vacancy, VKGL-NL_Rotterdam
-/. 2 - c.471C>T r.(?) p.(Tyr157=) - benign g.86012713C>T g.84252957C>T RGR(NM_002921.4):c.471C>T (p.Y157=) - RGR_000008 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen, VKGL-NL_Nijmegen
-?/. 1 4 c.474C>T r.(=) p.(=) - likely benign g.86012716C>T - c.474C>T - RGR_000034 - PubMed: Ksantini-2010 - - Germline - 46.0% of 216 patients - - - LOVD
?/. 1 - c.500C>T r.(?) p.(Thr167Ile) - VUS g.86012742C>T g.84252986C>T - - RGR_000015 - PubMed: Koyanagi 2019, Journal: Koyanagi 2019 - - Germline - 1/1204 cases with retinitis pigmentosa - - - Yoshito Koyanagi
?/. 3 - c.505G>T r.(?) p.(Asp169Tyr) - VUS g.86012747G>T g.84252991G>T RGR(NM_002921.3):c.505G>T (p.D169Y), RGR(NM_002921.4):c.505G>T (p.D169Y) - RGR_000018 VKGL data sharing initiative Nederland PubMed: Tiwari 2016 - - CLASSIFICATION record, Germline - - - - - VKGL-NL_Rotterdam, VKGL-NL_AMC
-?/. 1 - c.544T>C r.(?) p.(Phe182Leu) - likely benign g.86014101T>C g.84254345T>C RGR(NM_002921.3):c.544T>C (p.F182L) - RGR_000009 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.556_576del r.(?) p.(Phe186_Pro192del) - VUS g.86014113_86014133del - RGR(NM_002921.3):c.556_576delTTCTTCAACTTCGCCATGCCC (p.F186_P192del) - RGR_000038 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.642+10C>T r.(=) p.(=) - VUS g.86014209C>T g.84254453C>T - - RGR_000027 - PubMed: Xu 2014 - rs192139791 Germline - 1/314 case chromosomes - - - LOVD
-?/. 1 5i c.642+16G>A r.(?) p.? - likely benign g.86014215G>A - c.642+16G>A - RGR_000035 - PubMed: Ksantini-2010 - - Germline - 7.0% of 216 patients - - - LOVD
-?/. 1 - c.697A>C r.(?) p.(Ile233Leu) - likely benign g.86017703A>C g.84257947A>C RGR(NM_002921.3):c.697A>C (p.I233L) - RGR_000019 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/., -/. 4 6 c.734C>T r.(?) p.(Ser245Phe) - benign, likely pathogenic g.86017740C>T g.84257984C>T C734T, NM_001012720.1:c.454C>A - RGR_000016 - PubMed: Holtan 2020, PubMed: Koyanagi 2019, Journal: Koyanagi 2019, PubMed: Mandal 2005 - rs61730895 Germline - 208/1204 cases with retinitis pigmentosa, 7/899 cases, 9/1204 cases with retinitis pigmentosa - - - Global Variome, with Curator vacancy, Julia Lopez, Yoshito Koyanagi
+?/. 2 - c.734_735del r.(?) p.(Ser245Tyrfs*29) - VUS (!) g.86017740_86017741del g.84257984_84257985del RGR c.722_723delCC (p.S242Yfs*29) - RGR_000045 1 more item PubMed: Li 2016 - - Germline no - - - - LOVD
+?/., -/., -?/., ?/. 8 6i c.756+5A>G r.(spl?), r.spl, r.spl? p.(?), p.? - benign, likely benign, likely pathogenic, VUS g.86017767A>G g.84258011A>G c.756+5A>G, NM_001012720.1:c.454C>A, NM_001012720.1:c.744+5A>G, 1 more item - RGR_000001 predicted benign; not segregating with disease in other family, VKGL data sharing initiative Nederland PubMed: Booij-2011, PubMed: González-del Pozo-2011, PubMed: Holtan 2020, PubMed: Neveling 2012, 1 more item - rs143720091 CLASSIFICATION record, Germline no 1/899 cases - - - Global Variome, with Curator vacancy, Kornelia Neveling, VKGL-NL_Rotterdam, VKGL-NL_AMC
-/. 1 6i c.760-38C>T r.spl? p.? - benign g.86018229C>T - - - RGR_000030 - PubMed: Singh 2009 - - Germline - - - - - LOVD
-?/. 1 - c.762C>A r.(?) p.(Pro254=) - likely benign g.86018269C>A - RGR(NM_002921.3):c.762C>A (p.P254=) - RGR_000039 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.821A>G r.(?) p.(Glu274Gly) - VUS g.86018328A>G g.84258572A>G RGR(NM_002921.4):c.821A>G (p.E274G) - RGR_000020 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 1 - c.833_834insG r.(?) p.(Ile280Asnfs*78) - pathogenic (dominant) g.86018340_86018341insG - 10:86018339A>AG ENST00000359452.4:c.836dupG (Ile280AsnfsTer78) - RGR_000023 - PubMed: Carss 2017 - - Germline - - - - - LOVD
+/., +?/. 14 - c.836dup r.(?) p.(Ile280Asnfs*78) - likely pathogenic (dominant), pathogenic, pathogenic (dominant) g.86018343dup g.84258587dup NM_001012720.1:c.824dup (I276N*77), RGR 1-bp insertion in codon Gly275 (GGA?GGGA), 2 more items - RGR_000024 heterozygous, 1 more item PubMed: Ba-Abbad 2018, PubMed: Morimura 1999, PubMed: Turro 2020, PubMed: Wang 2017 - - Germline, Germline/De novo (untested) ?, yes 0/95 unaffected controls - - - LOVD
-?/. 1 7 c.*65A>G r.(=) p.(=) - likely benign g.86018460A>G - c.*65A>G - RGR_000036 - PubMed: Ksantini-2010 - - Germline - 11.0% of 216 patients - - - LOVD
-?/. 1 7_2 c.*100_*101insA r.(?) p.? - likely benign g.86018495_86018496insA - c.*100_101insA - RGR_000033 - PubMed: Ksantini-2010 - - Germline - 6.0% of 216 patients - - - LOVD
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