Full data view for gene TNXB

Ehlers Danlos Syndrome Variant Database


Information The variants shown are described using the NM_019105.6 transcript reference sequence.

6 entries on 1 page. Showing entries 1 - 6.
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Effect     

Exon     

AscendingDNA change (cDNA)     

RNA change     

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+/+ 8 c.3290_3291del r.(?) p.(Lys1097Argfs*48) frameshift deletion Both (homozygous) - pathogenic (recessive) g.32052345_32052346del g.32084568_32084569del delAA - TNXB_000001 - PubMed: Schalkwijk et al., 2001, PubMed: Voermans et al., 2007, PubMed: Voermans et al., 2007 - - Germline/De novo (untested) - - - - - DNA PCR, SEQ - - EDS Patient 1 PubMed: Schalkwijk et al., 2001, PubMed: Voermans et al., 2007, PubMed: Voermans et al., 2007 This patient's mother was deceased, but the patient's sister was heterozygous for the variant, with the maternal allele carrying the deletion - - - - - - - - 1 Raymond Dalgleish
+/+ 8 c.3290_3291del r.(?) p.(Lys1097Argfs*48) frameshift deletion Both (homozygous) - pathogenic (recessive) g.32052345_32052346del g.32084568_32084569del - - TNXB_000001 - PubMed: Schalkwijk et al., 2001 - - Germline/De novo (untested) - - - - - DNA PCR, SEQ - - EDS Patient 5 PubMed: Schalkwijk et al., 2001 This patient's parents were unavailable for study. - - - - - - - - 1 Raymond Dalgleish
+?/+? 8 c.3290_3291del r.(?) p.(Lys1097Argfs*48) frameshift deletion Parent #1 - likely pathogenic g.32052345_32052346del g.32084568_32084569del - - TNXB_000001 - PubMed: Demirdas et al., 2016 - - Unknown - - - - - DNA SEQ-NG, MLPA, SEQ - - EDS, EDSCLL Family VII PubMed: Demirdas et al., 2016 This patient was previously described as Patient 2 in {PMID 21959861:Hendriks et al., 2012}. The patient carried a TNXB/TNXA fusion gene from Parent#2 with a 30kb deletion encompassing CYP21A2. The fusion gene carried the described variants. The technique used was the custom NGS Gene panel. - - - - - - - - 1 Raymond Dalgleish
+/. - c.3290_3291del r.(?) p.(Lys1097ArgfsTer48) - - Unknown - pathogenic g.32052345_32052346del - TNXB(NM_019105.6):c.3290_3291delAA (p.(Lys1097fs)), TNXB(NM_019105.8):c.3290_3291delAA (p.K1097Rfs*48) - TNXB_000001 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - - - - - - - - - - - - - - - - - - -
+/. - c.3290_3291del r.(?) p.(Lys1097ArgfsTer48) - - Unknown - pathogenic g.32052345_32052346del - TNXB(NM_019105.6):c.3290_3291delAA (p.(Lys1097fs)), TNXB(NM_019105.8):c.3290_3291delAA (p.K1097Rfs*48) - TNXB_000001 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - - - - - - - - - - - - - - - - - - -
+/. - c.3290_3291del r.(?) p.(Lys1097ArgfsTer48) - - Unknown - pathogenic g.32052345_32052346del - TNXB(NM_019105.6):c.3290_3291delAA (p.(Lys1097fs)), TNXB(NM_019105.8):c.3290_3291delAA (p.K1097Rfs*48) - TNXB_000001 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - - - - - - - - - - - - - - - - - - -
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