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.

15 entries on 1 page. Showing entries 1 - 15.
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+/. - c.11432_11524+27del r.? p.? - - Parent #2 ACMG pathogenic (recessive) g.32011499_32011618del - - - TNXB_000025 - - - - Germline - - - - - DNA SEQ-NG-IT blood - EDSCLL Patient 9 - - F - Japan - - - - - 1 Tomoki Kosho
+/. - c.11435_11524+30del r.spl? p.? - - Unknown - pathogenic g.32011499_32011618del g.32043722_32043841del TNXB(NM_019105.8):c.11435_11524+30del - TNXB_000025 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - - - - - - - - - - - - - - - - - - -
+?/. 35 c.11435_11524+30del r.spl? p.? - - Paternal (inferred) - pathogenic g.32011499_32011618del g.32043722_32043841del - - TNXB_000025 - - - - Germline/De novo (untested) - - - - - DNA SEQ-NG blood - EDSCLL individual2 - - F - Italy - 26y - - - 1 Lucia Micale
+?/+? 35i c.11435_11524+30del r.spl p.? splicing affected? deletion Paternal (confirmed) - likely pathogenic g.32011496_32011615del - - - TNXB_000025 - PubMed: Micale et al., 2019 - - Unknown - - - - - DNA SEQ-NG, MLPA, PCR, SEQ - - EDS, EDSCLL Patient 2 PubMed: Micale et al., 2019 The patient carried a c.1150dupG variant and a pseudogene(TNXA)-derived 120bps deletion, likely the result of the formation of a chimeric TNXA/TNXB fusion gene. The technique used was the custom NGS Gene panel. - - Italy Italian - - - - 1 Raymond Dalgleish
-/- 35i c.11435_11524+30del r.spl p.? splicing affected? deletion Parent #1 - likely benign g.32011496_32011615del - - - TNXB_000025 - PubMed: Demirdas et al., 2016 - - Unknown - - - - - DNA SEQ-NG, MLPA, SEQ - - EDS, EDSCLL Family II PubMed: Demirdas et al., 2016 This patient was previously described in PubMed: Schalkwijk et al., 2001 as Patient 2. There are two other siblings carrying the same variant and phenotype. The variants are TNXA-derived, probably due to the formation of a chimeric gene. The technique used was the custom NGS Gene panel. - - - - - - - - 1 Raymond Dalgleish
-/- 35i c.11435_11524+30del r.spl p.? splicing affected? deletion Both (homozygous) - likely benign g.32011496_32011615del - - - TNXB_000025 - PubMed: Demirdas et al., 2016 - - Unknown - - - - - DNA SEQ-NG, MLPA, SEQ - - EDS, EDSCLL Family IV PubMed: Demirdas et al., 2016 This family was previously described in PubMed: Voermans et al., 2009. The patient carried a TNXB/TNXA fusion gene on the allele for Parent #1, causing a 30kb deletion encompassing CYP21A2. The fusion gene carried the described variants. The technique used was the custom NGS Gene panel. - - - - - - - - 1 Raymond Dalgleish
-/- 35i c.11435_11524+30del r.spl p.? splicing affected? deletion Parent #2 - likely benign g.32011496_32011615del - - - TNXB_000025 - 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
-/- 35i c.11435_11524+30del r.spl p.? splicing affected? deletion Parent #2 - likely benign g.32011496_32011615del - - - TNXB_000025 - PubMed: Demirdas et al., 2016 - - Unknown - - - - - DNA SEQ-NG, MLPA, SEQ - - EDS, EDSCLL Family VIII PubMed: Demirdas et al., 2016 The patient carried a TNXB/TNXA fusion gene with a 30kb deletion encompassing CYP21A2 on the allele from Parent #2. The technique used was the custom NGS Gene panel. - - - - - - - - 1 Raymond Dalgleish
-/- 35i c.11435_11524+30del r.spl p.? splicing affected? deletion Parent #2 - likely benign g.32011496_32011615del - - - TNXB_000025 - PubMed: Demirdas et al., 2016 - - Unknown - - - - - DNA SEQ-NG, MLPA, SEQ - - EDS, EDSCLL Family IX PubMed: Demirdas et al., 2016 The technique used was the custom NGS Gene panel. - - - - - - - - 1 Raymond Dalgleish
+?/+? 35i c.11435_11524+30del r.spl p.? splicing affected? deletion Unknown - likely pathogenic g.32011496_32011615del - - - TNXB_000025 - PubMed: Lao et al., 2019 - - Unknown - - - - - DNA PCR, SEQ - - adrenal hyperplasia, EDS - PubMed: Lao et al., 2019 26 patients with congenital adrenal hyperplasia showed evidence of the CAH-X CH-1 genotype, a chimeric TNXA/TNXB gene featuring the described variant. Up to 24 patients had a complete or partial clinical evaluation for EDS. - - - - - - - - 1 Raymond Dalgleish
+/. - c.11435_11524+30del r.? p.? deletion - Both (homozygous) ACMG pathogenic (recessive) g.32011499_32011618del g.32043722_32043841del - - TNXB_000025 - - - - Germline - - - - - DNA SEQ-NG-IT blood - EDSCLL Patient 2 - - M - Japan - - - - - 1 Tomoki Kosho
+/. - c.11435_11524+30del r.? p.? deletion - Parent #1 - pathogenic (recessive) g.32011499_32011618del g.32043722_32043841del - - TNXB_000025 - - - - Germline - - - - - DNA SEQ-NG-IT blood - EDSCLL Patient 7 - - M - Japan - - - - - 1 Tomoki Kosho
+/. - c.11435_11524+30del r.? p.? - - Maternal (confirmed) - pathogenic (recessive) g.32011499_32011618del g.32043722_32043841del - - TNXB_000025 - - - - Germline - - - - - DNA SEQ-NG-IT blood - EDSCLL Patient 4 - - F - Japan - - - - - 1 Tomoki Kosho
+/. - c.11435_11524+30del r.? p.? - - Paternal (confirmed) ACMG pathogenic (recessive) g.32011499_32011618del g.32043722_32043841del - - TNXB_000025 - - - - Germline - - - - - DNA SEQ-NG-IT blood - EDSCLL Patient 5 - - F - Japan - - - - - 1 Tomoki Kosho
+/. - c.11435_11524+30del r.? p.? deletion - Paternal (confirmed) ACMG pathogenic (recessive) g.32011499_32011618del - - - TNXB_000025 - - - - Germline - - - - - DNA SEQ-NG-IT blood - EDSCLL Patient 8 - - F - Japan - - - - - 1 Tomoki Kosho
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