All variants in the PPIB gene


Osteogenesis Imperfecta Variant Database
Information The variants shown are described using the NM_000942.4 transcript reference sequence.

28 entries on 1 page. Showing entries 1 - 28.
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Effect     

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     
-?/. - c.-3097del r.(?) p.(=) - likely benign g.64458294del g.64166095del CSNK1G1(NM_022048.4):c.*5848delT - CSNK1G1_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-?/. - c.-3097del r.(?) p.(=) - likely benign g.64458294del - CSNK1G1(NM_022048.5):c.*5848delT - CSNK1G1_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
+/+ 1 c.25A>G r.(?) p.(Met9Val) - pathogenic g.64455161T>C - - - PPIB_000016 - PubMed: Jiang 2017 - - Germline - - - - - Raymond Dalgleish
+/+ 1 c.25A>G r.(?) p.(Met9Val) - pathogenic g.64455161T>C - - - PPIB_000016 - PubMed: Caparros-Martin 2016 - - Germline - - - - - Raymond Dalgleish
+/+ 1 c.26T>G r.(?) p.(Met9Arg) - pathogenic g.64455160A>C - c.2T>G - PPIB_000011 - PubMed: Barnes 2010 - - Germline - - NlaIII- - - Raymond Dalgleish
-/. - c.63C>A r.(?) p.(Ser21=) - benign g.64455123G>T g.64162924G>T PPIB(NM_000942.5):c.63C>A (p.S21=) - PPIB_000010 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-/- 1 c.63C>A r.(?) p.(Ser21=) - benign g.64455123G>T - - - PPIB_000010 variant has been found in OI patients and in normal controls PubMed: Barbirato 2015 - rs4904 Germline - - - - - Raymond Dalgleish
-?/. - c.85G>A r.(?) p.(Gly29Arg) - likely benign g.64455101C>T g.64162902C>T PPIB(NM_000942.5):c.85G>A (p.G29R) - PPIB_000019 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
+/+ 1 c.120del r.(?) p.(Val42Serfs*16) - pathogenic g.64455066del - c.120delC - PPIB_000004 The deletion (maternal allele) leads to NMD of the mRNA and the substitution (paternal allele) destabilises the protein. PubMed: Pyott 2011 - - Germline - - - - - Peter Byers
?/. - c.175C>T r.(?) p.(Arg59Trp) - VUS g.64454314G>A - PPIB(NM_000942.5):c.175C>T (p.(Arg59Trp)) - CSNK1G1_000014 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/- 2 c.178G>T r.(?) p.(Val60Leu) - VUS g.64454311C>A - - - PPIB_000009 - - - rs11558595 Germline - - - - - Raymond Dalgleish
-?/. - c.249+12G>A r.(=) p.(=) - likely benign g.64454228C>T - PPIB(NM_000942.5):c.249+12G>A - CSNK1G1_000006 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-/. - c.249+19G>T r.(=) p.(=) - benign g.64454221C>A - PPIB(NM_000942.5):c.249+19G>T - CSNK1G1_000011 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
+/+ 3 c.302T>A r.(?) p.(Met101Lys) - pathogenic g.64452344A>T - - - PPIB_000014 - PubMed: Stephen 2015 - - Germline - - - - - Raymond Dalgleish
+/+ 2 c.313G>A r.(?) p.(Gly105Arg) - pathogenic g.64452333C>T - - - PPIB_000005 The deletion (maternal allele) leads to NMD of the mRNA and the substitution (paternal allele) destabilises the protein. PubMed: Pyott 2011 - - Germline - - - - - Peter Byers
-/. - c.324C>T r.(?) p.(Thr108=) - benign g.64452322G>A g.64160123G>A PPIB(NM_000942.5):c.324C>T (p.T108=) - PPIB_000008 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
?/- 3 c.324C>T r.(?) p.(=) - VUS g.64452322G>A - - - PPIB_000008 - - - rs2307247 Germline - - - - - Raymond Dalgleish
+/+ 3i c.343+1G>A r.spl p.? - pathogenic g.64452302C>T - - - PPIB_000006 The variant leads to retention of 27 bases of intron 3 in the stable RNA and also to exon 3 skipping (unstable). PubMed: Pyott 2011 - - Germline - - - - - Peter Byers
-?/- 3i c.344-27C>T r.(?) p.(=) - likely benign g.64449135G>A - - - PPIB_000015 absent in 100 control samples PubMed: Barbirato 2015 - - Germline - - - - - Raymond Dalgleish
+/+ 4 c.414_423del r.(?) p.(Ser139Thrfs*21) - pathogenic g.64449029_64449038del - - - PPIB_000003 - PubMed: Pyott 2011 - - Germline - - - - - Peter Byers
+/. - c.451C>T r.(?) p.(Gln151*) - pathogenic (recessive) g.64449001G>A g.64156802G>A NM_000942.4:c.451C>T:p.(Gln151*) - PPIB_000001 - PubMed: Maddirevula 2018 - - Germline - - - - - LOVD
+/+ 4 c.451C>T r.(?) p.(Gln151*) - pathogenic g.64449001G>A - - - PPIB_000001 - PubMed: Van Dijk 2009 - - Germline - - - - - Raymond Dalgleish
?/- 4 c.492C>G r.(?) p.(=) - VUS g.64448960G>C - - - PPIB_000007 - - - rs28720193 Germline - - - - - Raymond Dalgleish
+/+ 4 c.497A>C r.(?) p.(His166Pro) - pathogenic g.64448955T>G - - - PPIB_000013 - PubMed: Caparrós-Martin 2013 - - Germline - - - - - Victor L Ruiz-Perez
+/+ 4 c.509G>A r.(?) p.(Gly170Asp) - pathogenic g.64448943C>T - - - PPIB_000017 - PubMed: Jiang 2017 - - Germline - - - - - Raymond Dalgleish
+/+ 5 c.556_559del r.(?) p.(Lys186Glnfs*8) - pathogenic g.64448314_64448317del - - - PPIB_000002 - PubMed: van Dijk 2009 - - Germline - - - - - Raymond Dalgleish
+?/+ 5 c.563_566del r.(?) p.(Asp188Alafs*6) - likely pathogenic g.64448307_64448310del - - - PPIB_000012 - PubMed: Barnes 2012 - - Germline - - - - - Aileen Barnes
?/. - c.646del r.(?) p.(Glu216SerfsTer58) - VUS g.64448228del - PPIB(NM_000942.4):c.646delG (p.E216Sfs*58) - PPIB_000018 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
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