Unique variants in the MBTPS2 gene


Osteogenesis Imperfecta Variant Database
Information The variants shown are described using the NM_015884.3 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 1 c.-262C>A r.(?) p.? - VUS g.21857591C>A g.21839473C>A - - MBTPS2_000002 - - - rs6528055 Germline - 0.14-0.39 - - - Emmelien Aten
+/. 1 - c.2T>C r.(2T>C) p.(Met1?) - pathogenic (recessive) g.21857854T>C g.21839736T>C - - MBTPS2_000096 - PubMed: Chen 2020 - - De novo - - - - - Johan den Dunnen
-?/. 2 - c.30G>C r.(?) p.(Val10=) - likely benign g.21857882G>C - MBTPS2(NM_015884.3):c.30G>C (p.V10=), MBTPS2(NM_015884.4):c.30G>C (p.V10=) - MBTPS2_000075 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_VUmc
+/. 1 - c.71T>C r.(?) p.(Leu24Pro) - pathogenic (recessive) g.21857923T>C g.21839805T>C - - MBTPS2_000093 - PubMed: Izumi 2013 - - Germline - - - - - Johan den Dunnen
?/. 2 1i c.76-328G>C r.(=), r.(?) p.(=) - VUS g.21860960G>C g.21842842G>C - - MBTPS2_000003 - - - rs6528056 Germline - 0.24 - - - Yu Sun, Emmelien Aten
+/. 1 - c.154T>A r.(?) p.(Trp52Arg) - pathogenic (recessive) g.21861366T>A g.21843248T>A - - MBTPS2_000098 - PubMed: Khan 2019 - - Germline yes - - - - Johan den Dunnen
?/. 1 2 c.197G>C r.(?) p.(Arg66Pro) - VUS g.21861409G>C g.21843291G>C - - MBTPS2_000004 - - - rs5951639 Germline - 0.00 - - - Emmelien Aten
-?/. 1 - c.217T>A r.(?) p.(Tyr73Asn) - likely benign g.21861429T>A - MBTPS2(NM_015884.3):c.217T>A (p.Y73N) - MBTPS2_000077 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/., -/., ?/. 5 2 c.222A>G r.(?) p.(=), p.(Gln74=) - benign, likely pathogenic, VUS g.21861434A>G g.21843316A>G - - MBTPS2_000005 association with plasma HDLC levels (women) PubMed: Lu 2008 - rs3213451 Germline, Unknown - 0.24-0.57 - - - Yu Sun, Emmelien Aten
?/. 1 2i c.224+51G>A r.(?) p.(=) - VUS g.21861487G>A g.21843369G>A - - MBTPS2_000006 - - - rs3213452 Germline - 0.00-0.005 - - - Emmelien Aten
?/. 1 2i c.225-371G>A r.(?) p.(=) - VUS g.21862918G>A g.21844800G>A - - MBTPS2_000007 - - - rs7057211 Germline - 0.00-0.002 - - - Emmelien Aten
-?/. 1 - c.225-267G>A r.(=) p.(=) - likely benign g.21863022G>A - MBTPS2(NM_015884.4):c.225-267G>A - MBTPS2_000082 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
+?/. 1 - c.245T>C r.(?) p.(Phe82Ser) - likely pathogenic (recessive) g.21863309T>C g.21845191T>C - - MBTPS2_000101 - PubMed: Shin 2022 - - Germline - - - - - Johan den Dunnen
+/. 3 2i c.225-6T>A r.224_225ins225-4_225-1, r.spl p.?, p.Trp75* - pathogenic g.21863283T>A g.21845165T>A c.225-6A, c.225-6A/WT - MBTPS2_000022 not in 225 control chromosomes, 1 more item PubMed: Oeffner 2009, PubMed: Oeffner 2009, PubMed: Oeffner 2011 - - Germline - - - - - Emmelien Aten
+/., +?/., -/. 16 3 c.261G>A r.(?) p.(Met87Ile), p.Met87Ile - likely pathogenic, NA, pathogenic g.21863325G>A g.21845207G>A c.261A, c.261A/WT - MBTPS2_000018 cloned in EYFP fusion construc in pcDNA3.1; CHO expression limited complementation SP2 deficiency, 5 more items PubMed: Ming 2009, PubMed: Oeffner 2009, PubMed: Oeffner 2009; PubMed: Bornholdt 2013 - - Germline, In vitro (cloned), Unknown - - - - - Emmelien Aten, Karl-Heinz Grzeschik
-?/. 1 - c.291G>A r.(?) p.(Thr97=) - likely benign g.21863355G>A g.21845237G>A MBTPS2(NM_015884.3):c.291G>A (p.T97=) - MBTPS2_000050 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.307G>A r.(?) p.(Ala103Thr) - likely benign g.21863371G>A g.21845253G>A MBTPS2(NM_015884.3):c.307G>A (p.A103T) - MBTPS2_000061 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.330C>G r.(?) p.(Pro110=) - likely benign g.21863394C>G - MBTPS2(NM_015884.3):c.330C>G (p.P110=) - MBTPS2_000078 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/., ?/. 2 3 c.338A>C r.(?) p.(Tyr113Ser) - likely benign, VUS g.21863402A>C g.21845284A>C MBTPS2(NM_015884.4):c.338A>C (p.(Tyr113Ser)) - MBTPS2_000008 VKGL data sharing initiative Nederland - - rs7059508 CLASSIFICATION record, Germline - 0.00 - - - Emmelien Aten, VKGL-NL_Leiden
?/. 1 3 c.339C>T r.(?) p.(=) - VUS g.21863403C>T g.21845285C>T - - MBTPS2_000009 1 more item - - rs7063422 Germline - 0.00 - - - Emmelien Aten
-?/., ?/. 4 - c.366_383del r.(?) p.(Ser131_Ser136del) - likely benign, VUS g.21863430_21863447del g.21845312_21845329del 1 more item - MBTPS2_000051 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_VUmc
?/. 1 3 c.387_389del Ser130del p.(Ser136del) - VUS g.21863451_21863453del g.21845333_21845335del - - MBTPS2_000010 - - - rs3834687 Germline - - - - - Emmelien Aten
-?/. 2 - c.390_392del r.(?) p.(Ser136del) - likely benign g.21863454_21863456del g.21845336_21845338del MBTPS2(NM_015884.3):c.390_392del (p.(Ser136del)), MBTPS2(NM_015884.3):c.390_392delATC (p.S136del) - MBTPS2_000052 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam
-?/. 1 - c.408G>A r.(?) p.(Ser136=) - likely benign g.21863472G>A g.21845354G>A MBTPS2(NM_015884.3):c.408G>A (p.S136=) - MBTPS2_000053 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 3i c.438+59A>C r.(?) p.(=) - VUS g.21863561A>C g.21845443A>C - - MBTPS2_000011 - - - rs7063399 Germline - 0.00-0.08 - - - Emmelien Aten
-/., -?/. 2 - c.459C>T r.(?) p.(Pro153=) - benign, likely benign g.21869647C>T g.21851529C>T MBTPS2(NM_015884.3):c.459C>T (p.P153=), MBTPS2(NM_015884.4):c.459C>T (p.P153=) - MBTPS2_000054 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_VUmc
-?/. 1 - c.460G>A r.(?) p.(Val154Ile) - likely benign g.21869648G>A - MBTPS2(NM_015884.3):c.460G>A (p.V154I) - MBTPS2_000079 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/., ?/. 3 - c.485C>T r.(?) p.(Thr162Met) - likely benign, VUS g.21869673C>T g.21851555C>T MBTPS2(NM_015884.3):c.485C>T (p.T162M, p.(Thr162Met)), MBTPS2(NM_015884.4):c.485C>T (p.T162M) - MBTPS2_000055 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Groningen
+?/. 1 - c.516A>C r.(?) p.(Glu172Asp) - likely pathogenic (recessive) g.21869704A>C g.21851586A>C - - MBTPS2_000094 - PubMed: Lim 2023 - - Germline - - - - - Johan den Dunnen
?/. 3 4i c.542+253A>G r.(=), r.(?) p.(=) - VUS g.21869983A>G g.21851865A>G - - MBTPS2_000012 - - - rs2071210 Germline - 0.12-0.20 - - - Yu Sun, Emmelien Aten
+?/. 1 - c.599C>T r.(?) p.(Ala200Val) - likely pathogenic (recessive) g.21871550C>T g.21853432C>T - - MBTPS2_000088 - PubMed: Zhang 2016, Journal: Zhang 2016 - - Germline/De novo (untested) - - - - - Johan den Dunnen
-?/. 1 - c.612G>A r.(?) p.(Leu204=) - likely benign g.21871563G>A - MBTPS2(NM_015884.3):c.612G>A (p.L204=) - MBTPS2_000080 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 2 - c.638C>G r.(?) p.(Ser213Trp) - likely pathogenic, likely pathogenic (dominant) g.21871589C>G g.21853471C>G - - MBTPS2_000086 VKGL data sharing initiative Nederland PubMed: Cuperus 2025, Journal: Cuperus 2025 - - CLASSIFICATION record, De novo - - - - - Johan den Dunnen, VKGL-NL_Nijmegen
+/. 1 - c.638C>T r.(?) p.(Ser213Leu) - pathogenic (recessive) g.21871589C>T g.21853471C>T C638T - MBTPS2_000087 - PubMed: Chen 2019, Journal: Chen 2019 - - Germline yes - - - - Johan den Dunnen
-?/. 1 - c.639G>A r.(?) p.(Ser213=) - likely benign g.21871590G>A g.21853472G>A MBTPS2(NM_015884.3):c.639G>A (p.S213=) - MBTPS2_000063 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 2 - c.670+2766T>C r.(=) p.(=) - VUS g.21874387T>C g.21856269T>C - - MBTPS2_000039 - - - - Germline - - - - - Yu Sun
?/. 1 - c.670+2842del r.(=) p.(=) - VUS g.21874463del - MBTPS2(NM_015884.4):c.670+2842del - MBTPS2_000083 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.670+3004C>G r.(=) p.(=) - likely benign g.21874625C>G - YY2(NM_206923.3):c.23C>G (p.(Ser8Cys)) - MBTPS2_000084 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.670+3250A>G r.(=) p.(=) - likely benign g.21874871A>G g.21856753A>G YY2(NM_206923.3):c.269A>G (p.Y90C) - MBTPS2_000071 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.670+3313T>C r.(=) p.(=) - VUS g.21874934T>C - YY2(NM_206923.3):c.332T>C (p.(Ile111Thr)) - MBTPS2_000085 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.670+3888C>G r.(=) p.(=) - VUS g.21875509C>G g.21857391C>G YY2(NM_206923.3):c.907C>G (p.(Leu303Val)) - MBTPS2_000067 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.670+3993G>A r.(=) p.(=) - likely benign g.21875614G>A g.21857496G>A YY2(NM_206923.3):c.1012G>A (p.(Asp338Asn)) - MBTPS2_000069 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 2 - c.670+4027A>G r.(=) p.(=) - VUS g.21875648A>G g.21857530A>G YY2(NM_206923.3):c.1046A>G (p.N349S, p.(Asn349Ser)) - MBTPS2_000057 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam
?/. 2 - c.670+4200del r.(=) p.(=) - VUS g.21875821del g.21857703del - - MBTPS2_000049 - - - - Germline - - - - - Yu Sun
?/. 1 - c.670+4491A>G r.(=) p.(=) - VUS g.21876112A>G g.21857994A>G - - MBTPS2_000042 - - - - Germline - - - - - Yu Sun
+/. 3 5i c.671-9T>G r.(671_789del), r.671_789del p.(Ile225Leufs*25), p.Ile225Leufs*25 - pathogenic, pathogenic (recessive) g.21886576T>G g.21868458T>G - - MBTPS2_000028 patient RNA analysis and minigene construct PubMed: Oeffner 2011, PubMed: Wang 2013 - - Germline - - - - - Johan den Dunnen, Emmelien Aten
+/., +?/., -/. 4 6 c.677G>T r.(?) p.(Trp226Leu), p.Trp226Leu - NA, pathogenic g.21886591G>T g.21868473G>T c.677T - MBTPS2_000020 cloned in EYFP fusion construc in pcDNA3.1; CHO expression limited complementation SP2 deficiency, 3 more items PubMed: Oeffner 2009 - - Germline, In vitro (cloned) - - BstXI - - Emmelien Aten
+/., +?/., -/., -?/. 8 6 c.680A>T r.(?) p.(His227Leu), p.His227Leu - NA, pathogenic g.21886594A>T g.21868476A>T c.680T, c.680T/WT - MBTPS2_000001 cloned in EYFP fusion construc in pcDNA3.1; CHO expression limited complementation SP2 deficiency, 3 more items PubMed: Oeffner 2009 - - Germline, In vitro (cloned) - - - - - Emmelien Aten
+/., +?/. 5 6 c.686T>C r.(?) p.(Phe229Ser), Phe229Ser - likely pathogenic, pathogenic (recessive) g.21886600T>C g.21868482T>C Phe229Ser - MBTPS2_000029 Variant absent from 160 control X-chromosomes., variant not in mother PubMed: Bornholdt 2013, PubMed: Murase 2020 - - Germline, Germline/De novo (untested) - - - - X-inactivation not analysed Johan den Dunnen, Karl-Heinz Grzeschik
?/. 1 - c.706A>G r.(?) p.(Ile236Val) - VUS g.21886620A>G g.21868502A>G MBTPS2(NM_015884.3):c.706A>G (p.(Ile236Val)) - MBTPS2_000058 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. 1 - c.727C>T r.(?) p.(Pro243Ser) ACMG likely pathogenic (recessive) g.21886641C>T g.21868523C>T - - MBTPS2_000104 ACMG PM2, PP3, PS2 PubMed: Migliavacca 2023 - - De novo - - - - - Johan den Dunnen
+/., +?/. 3 6 c.758G>C r.(?) Gly253Ala, p.(Gly253Ala) ACMG likely pathogenic, pathogenic g.21886672G>C g.21868554G>C - - MBTPS2_000030 1 more item PubMed: Bornholdt 2013, PubMed: Stray-Pedersen 2017 - - Germline - - PstI - - Johan den Dunnen, Karl-Heinz Grzeschik
+?/. 1 - c.766G>T r.(?) p.(Val256Leu) - likely pathogenic (recessive) g.21886680G>T g.21868562G>T 766G>A (Val256Leu) - MBTPS2_000100 variant characterised functionally PubMed: Strong 2021 - - Germline - - - - - Johan den Dunnen
?/. 1 6 c.774C>G r.(?) p.Ile258Met - VUS g.21886688C>G g.21868570C>G - - MBTPS2_000033 Variant absent from 160 control X-chromosomes. PubMed: Bornholdt 2013 - - Unknown - - PagI - - Karl-Heinz Grzeschik
?/. 1 6i c.789+104C>T r.(?) p.(=) - VUS g.21886807C>T g.21868689C>T - - MBTPS2_000013 - - - rs6653655 Germline - 0.00-0.04 - - - Emmelien Aten
?/. 1 6i c.790-229C>T r.(?) p.(=) - VUS g.21887387C>T g.21869269C>T - - MBTPS2_000014 - - - rs2071191 Germline - 0.13-0.40 - - - Emmelien Aten
-?/. 1 - c.844C>T r.(?) p.(Leu282=) - likely benign g.21887670C>T - MBTPS2(NM_015884.3):c.844C>T (p.L282=) - MBTPS2_000081 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 2 7i c.970+5G>A r.951_970del, r.spl p.?, p.Gln317HisfsTer10 ACMG likely pathogenic (recessive), pathogenic (recessive) g.21887801G>A g.21869683G>A - - MBTPS2_000099 mii-gene splicing assay shows deletion ex7 in mRNA PubMed: Chen 2023, PubMed: Irurzun 2021 - - Germline - - - - - Johan den Dunnen
?/. 2 - c.970+1309C>T r.(=) p.(=) - VUS g.21889105C>T g.21870987C>T - - MBTPS2_000043 - - - - Germline - - - - - Yu Sun
+/. 1 8 c.1001G>A r.(?) p.(Cys334Tyr) - pathogenic (recessive) g.21896190G>A g.21878072G>A - - MBTPS2_000092 - PubMed: Pietrzak 2012 - - Germline - - - - - Johan den Dunnen
+?/. 1 - c.1004G>T r.(?) p.(Cys335Phe) - likely pathogenic (recessive) g.21896193G>T g.21878075G>T - - MBTPS2_000102 - PubMed: Caengprasath 2023 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.1061G>A r.(?) p.(Arg354His) - likely benign g.21896250G>A g.21878132G>A MBTPS2(NM_015884.4):c.1061G>A (p.R354H) - MBTPS2_000070 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-?/. 1 - c.1102A>T r.(?) p.(Thr368Ser) - likely benign g.21896651A>T - MBTPS2(NM_015884.3):c.1102A>T (p.T368S) - MBTPS2_000073 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/., -?/. 3 - c.1237C>T r.(?) p.(His413Tyr) - benign, likely benign g.21896786C>T g.21878668C>T 1 more item - MBTPS2_000059 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_VUmc
?/. 1 9i c.1261+103A>G r.(?) p.(=) - VUS g.21896913A>G g.21878795A>G - - MBTPS2_000015 - - - rs3752405 Germline - 0.03-0.33 - - - Emmelien Aten
?/. 1 9i c.1262-173C>A r.(?) p.(=) - VUS g.21898842C>A g.21880724C>A - - MBTPS2_000016 - - - rs6633469 Germline - 0.03-0.33 - - - Emmelien Aten
+/., +?/., -/. 24 10 c.1286G>A r.(?) p.(Arg429His), p.Arg429His - likely pathogenic, NA, pathogenic, pathogenic (recessive) g.21899039G>A g.21880921G>A c.1286A/WT, MBTPS2(NM_015884.4):c.1286G>A (p.R429H), R429H - MBTPS2_000019 1 heterozygous, no homozygous; Clinindb (India), not in 225 control chromosomes, 5 more items Schwartz 2009, Workshop Fragile-X and XLMR, A96, PubMed: Basilious 2020, PubMed: Corujeira 2013, 5 more items - rs122468178 CLASSIFICATION record, Germline, Germline/De novo (untested), In vitro (cloned), Unknown yes 1/2795 individuals TaiI - - Johan den Dunnen, Emmelien Aten, Karl-Heinz Grzeschik, VKGL-NL_Nijmegen, VKGL-NL_VUmc, Mohammed Faruq
+/. 1 - c.1298T>C r.(?) p.(Leu433Pro) - pathogenic (recessive) g.21899051T>C g.21880933T>C - - MBTPS2_000097 - PubMed: Jiang 2019 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.1360G>C r.(?) p.(Ala454Pro) - pathogenic (recessive) g.21900573G>C g.21882455G>C - - MBTPS2_000090 - PubMed: Araujo 2015 - - Germline/De novo (untested) - - - - - Johan den Dunnen
?/. 1 - c.1370T>C r.(?) p.(Ile457Thr) - VUS g.21900583T>C - MBTPS2(NM_015884.4):c.1370T>C (p.I457T) - MBTPS2_000074 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+/., +?/. 3 11 c.1376A>G r.(?) p.(Asn459Ser) - likely pathogenic, pathogenic, pathogenic (recessive) g.21900589A>G g.21882471A>G - - MBTPS2_000040 ACMG, variant not in 644 control chromosomes; skewed inactivation in female carriers PubMed: Lindert 2016, Journal: Lindert 2016, PubMed: Lindert 2016, PubMed: Lim 2021, 1 more item - - De novo, Germline yes - - - - Johan den Dunnen, Cecilia Giunta
+?/. 1 11 c.1388G>T r.(?) p.(Cys463Phe) - likely pathogenic g.21900601G>T g.21882483G>T - - MBTPS2_000027 - - - - Germline - - - - - Emmelien Aten
+/. 1 - c.1391T>C r.(?) p.(Phe464Ser) - pathogenic (recessive) g.21900604T>C g.21882486T>C - - MBTPS2_000089 - PubMed: Haghighi 2013 - - Germline yes - - - - Johan den Dunnen
-?/. 1 - c.1407A>G r.(?) p.(Gln469=) - likely benign g.21900620A>G g.21882502A>G MBTPS2(NM_015884.3):c.1407A>G (p.Q469=) - MBTPS2_000072 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/., +?/., -/. 7 11 c.1424T>C r.(?) p.(Phe475Ser), p.Phe475Ser - likely pathogenic, NA, pathogenic, pathogenic (recessive) g.21900637T>C g.21882519T>C c.1424C - MBTPS2_000021 cloned in EYFP fusion construc in pcDNA3.1; CHO expression no complementation SP2 deficiency, 4 more items PubMed: Nemer 2017, PubMed: Oeffner 2009, PubMed: Oeffner 2009; PubMed: Bornholdt 2013 - - Germline, In vitro (cloned) yes - Eco130i, Eco130L - - Johan den Dunnen, Emmelien Aten, Karl-Heinz Grzeschik
+?/. 3 11 c.1427T>C r.(?) Leu476Ser - likely pathogenic g.21900640T>C g.21882522T>C - - MBTPS2_000031 1 more item PubMed: Bornholdt 2013 - - Germline, Unknown - - - - - Karl-Heinz Grzeschik
+?/. 1 11 c.1430A>T r.(?) p.Asp477Val - likely pathogenic g.21900643A>T g.21882525A>T - - MBTPS2_000032 1 more item PubMed: Bornholdt 2013 - - Unknown - - - - - Karl-Heinz Grzeschik
+/+, +/. 2 11 c.1433C>A r.(?) p.(Ala478Asp) - pathogenic, pathogenic (recessive) g.21900646C>A g.21882528C>A - - MBTPS2_000026 - PubMed: Ferrari 2017, PubMed: Tang 2011 - - Germline/De novo (untested), Unknown - - - - - Johan den Dunnen, Emmelien Aten
+/. 1 11 c.1468A>G r.(?) p.(Lys490Glu) - pathogenic g.21900681A>G g.21882563A>G - - MBTPS2_000023 - Schwartz 2009, Workshop Fragile-X and XLMR, A96 - - Unknown - - - - - Emmelien Aten
+/. 1 - c.1494G>T r.(?) p.(Leu498Phe) - pathogenic (recessive) g.21900707G>T g.21882589G>T - - MBTPS2_000095 - PubMed: Yang 2019 - - Germline - - - - - Johan den Dunnen
+/., +?/. 8 11 c.1499G>A r.(?) p.(Gly500Asp), p.Gly500Asp - likely pathogenic, likely pathogenic (recessive), pathogenic g.21900712G>A g.21882594G>A MBTPS2(NM_015884.3):c.1499G>A (p.G500D) - MBTPS2_000024 VKGL data sharing initiative Nederland, 1 more item Schwartz 2009, Workshop Fragile-X and XLMR, A96, PubMed: Bornholdt 2013, PubMed: Rubaian 2023 - - CLASSIFICATION record, Germline, Unknown - - - - - Johan den Dunnen, Emmelien Aten, VKGL-NL_Rotterdam, Karl-Heinz Grzeschik, VKGL-NL_Nijmegen
+/. 3 11 c.1515G>C r.(?) p.(Leu505Phe) - pathogenic, pathogenic (recessive) g.21900728G>C g.21882610G>C - - MBTPS2_000041 - PubMed: Lindert 2016, Journal: Lindert 2016, PubMed: Lindert 2016, PubMed: Lim 2021 - - Germline yes - - - - Johan den Dunnen, Cecilia Giunta
+?/. 1 - c.1523A>C r.(?) p.(Asn508Thr) - likely pathogenic (recessive) g.21900736A>C g.21882618A>C - - MBTPS2_000091 - PubMed: Fong 2015 - - Germline - - - - - Johan den Dunnen
+/., +?/. 10 11 c.1523A>G r.(?), r.1523a>g p.(Asn508Ser), p.Asn508Ser - likely pathogenic, NA, pathogenic, pathogenic (recessive) g.21900736A>G g.21882618A>G - - MBTPS2_000025 cloned in EYFP fusion construc in pcDNA3.1; CHO expression no complementation SP2 deficiency, 4 more items PubMed: Aten 2010, PubMed: Aten 2010; PubMed: Bornholdt 2013, PubMed: Ding 2010, PubMed: Fong 2012 - - CLASSIFICATION record, Germline, In vitro (cloned), Unknown yes - BsrI - - Johan den Dunnen, Emmelien Aten, Karl-Heinz Grzeschik, VKGL-NL_Nijmegen
+?/. 1 - c.1523A>T r.(?) p.(Asn508Ile) - likely pathogenic (recessive) g.21900736A>T g.21882618A>T - - MBTPS2_000103 - PubMed: Caengprasath 2023 - - Germline - - - - - Johan den Dunnen
+?/. 1 - c.1534G>A r.(?) p.(Gly512Arg) - VUS g.21900747G>A - - - MBTPS2_000076 - - - - Germline/De novo (untested) - - UA-01-2021 - - Lidiia Zhytnik
+?/. 2 11 c.1538T>C r.(?) p.(Leu513Pro), p.Leu513Pro ACMG likely pathogenic, likely pathogenic (recessive) g.21900751T>C g.21882633T>C - - MBTPS2_000034 ACMG PM1(sp), PM2(m), PP3(m), PP5(sp), Variant absent from 160 control X-chromosomes. PubMed: Bornholdt 2013, PubMed: Plachy 2019, PubMed: Plachy 2023 - - Germline/De novo (untested), Unknown - - EcoO109I - - Johan den Dunnen, Karl-Heinz Grzeschik
?/. 3 11 c.*1130G>A r.(=), r.(?) p.(=) - VUS g.21901903G>A g.21883785G>A - - MBTPS2_000017 - - - rs5951476 Germline - 0.22-0.56 - - - Yu Sun, Emmelien Aten
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