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Cardiac arrhythmias - additional genes v3.7 TANGO2 Achchuthan Shanmugasundram Publications for gene: TANGO2 were set to 26805781; 26805782; 30245509; 31339582; 32929747
Cardiac arrhythmias - additional genes v3.6 TANGO2 Achchuthan Shanmugasundram changed review comment from: Comment on list classification: There are over 20 unrelated patients reported with biallelic TANGO2 variants and with cardiac arrhythmia as one of the clinical presentations of the TANGO2-related disorder.

Expert review is being sought on the promotion of this gene to green rating on this panel. This is due to this gene being not previously approved for promotion to green rating on this panel by the NHS Genomic Medicine Service.; to: Comment on list classification: There are over 30 unrelated patients reported with biallelic TANGO2 variants and with cardiac arrhythmia as one of the clinical presentations of the TANGO2-related disorder.

Expert review is being sought on the promotion of this gene to green rating on this panel. This is due to this gene being not previously approved for promotion to green rating on this panel by the NHS Genomic Medicine Service.
Cardiac arrhythmias - additional genes v3.6 TANGO2 Achchuthan Shanmugasundram edited their review of gene: TANGO2: Changed publications to: 26805781, 26805782, 30245509, 31339582, 32929747, 35568137, 40156300
Cardiac arrhythmias - additional genes v3.6 TANGO2 Achchuthan Shanmugasundram changed review comment from: PMID:26805781 (2016) reported the identification of biallelic variants (a missense variant and two different intragenic deletions) in 12 patients from nine unrelated families with episodic rhabdomyolysis, hypoglycemia, hyperammonemia, and susceptibility to life-threatening cardiac tachyarrhythmias. Life-threatening cardiac tachyarrhythmia presented as torsade de pointes or ventricular tachycardia in 4 of 12 patients.

PMID:26805782 (2016) reported the identification of three different biallelic truncating variants in TANGO2 genes in three unrelated patients with infantile-onset metabolic disorder characterised by encephalopathy, hypoglycemia, rhabdomyolysis, arrhythmias, and laboratory findings suggestive of a defect in mitochondrial fatty acid oxidation.

PMID:30245509 (2018) reported the identification of biallelic TANGO2 variants (five different variants including the previously described exon 3-9 intragenic deletion in either homozygous or compound heterozygous states) in 14 individuals from 11 unrelated families with a complex clinical phenotype including primarily neurological presentations. Arrhythmia was reported in five of 14 patients.

PMID:31339582 (2020) reported nine patients from seven unrelated families with biallelic TANGO2 variants (exons 3-9 deletion and small variants). All, but one patient showed cardiac arrhythmias.

PMID:32929747 (2021) conducted a retrospective analysis of patients with a diagnosis of TANGO2 disease, where seven single nucleotide variants (five were novel), 2 small deletions and exons 3-9 deletion were identified in 20 patients from 14 families. 12 of these patients had cardiac abnormalities - long QT in ten, Brugada pattern in two, and cardiac arrhythmia in six.; to: PMID:26805781 (2016) reported the identification of biallelic variants (a missense variant and two different intragenic deletions) in 12 patients from nine unrelated families with episodic rhabdomyolysis, hypoglycemia, hyperammonemia, and susceptibility to life-threatening cardiac tachyarrhythmias. Life-threatening cardiac tachyarrhythmia presented as torsade de pointes or ventricular tachycardia in 4 of 12 patients.

PMID:26805782 (2016) reported the identification of three different biallelic truncating variants in TANGO2 genes in three unrelated patients with infantile-onset metabolic disorder characterised by encephalopathy, hypoglycemia, rhabdomyolysis, arrhythmias, and laboratory findings suggestive of a defect in mitochondrial fatty acid oxidation.

PMID:30245509 (2018) reported the identification of biallelic TANGO2 variants (five different variants including the previously described exon 3-9 intragenic deletion in either homozygous or compound heterozygous states) in 14 individuals from 11 unrelated families with a complex clinical phenotype including primarily neurological presentations. Arrhythmia was reported in five of 14 patients.

PMID:31339582 (2020) reported nine patients from seven unrelated families with biallelic TANGO2 variants (exons 3-9 deletion and small variants). All, but one patient showed cardiac arrhythmias.

PMID:32929747 (2021) conducted a retrospective analysis of patients with a diagnosis of TANGO2 disease, where seven single nucleotide variants (five were novel), 2 small deletions and exons 3-9 deletion were identified in 20 patients from 14 families. 12 of these patients had cardiac abnormalities - long QT in ten, Brugada pattern in two, and cardiac arrhythmia in six.

PMID:35568137 (2022) conducted a retrospective multicentre chart review of TANGO2 deficiency disorder patients admitted with cardiac crises, where 27 children were admitted for 43 cardiac crises at 14 centres. Arrhythmias included ventricular tachycardia in 21 (78%), supraventricular tachycardia in 3 (11%), and heart block in 1 (4%). Nineteen patients (70%) developed cardiomyopathy, and 20 (74%) experienced a cardiac arrest.

PMID:40156300 (2025) reported two unrelated patients with biallelic TANGO2 pathogenic variants (homozygous deletion of exons 4-6 in one and c.605+1G>A in the other). Both patients developed ventricular tachyarrhythmias, and the echocardiogram showed cardiomyopathy.
Cardiac arrhythmias - additional genes v3.6 TANGO2 Achchuthan Shanmugasundram Tag Q3_25_promote_green tag was added to gene: TANGO2.
Tag Q3_25_expert_review tag was added to gene: TANGO2.
Cardiac arrhythmias - additional genes v3.6 TANGO2 Achchuthan Shanmugasundram Classified gene: TANGO2 as Amber List (moderate evidence)
Cardiac arrhythmias - additional genes v3.6 TANGO2 Achchuthan Shanmugasundram Added comment: Comment on list classification: There are over 20 unrelated patients reported with biallelic TANGO2 variants and with cardiac arrhythmia as one of the clinical presentations of the TANGO2-related disorder.

Expert review is being sought on the promotion of this gene to green rating on this panel. This is due to this gene being not previously approved for promotion to green rating on this panel by the NHS Genomic Medicine Service.
Cardiac arrhythmias - additional genes v3.6 TANGO2 Achchuthan Shanmugasundram Gene: tango2 has been classified as Amber List (Moderate Evidence).
Cardiac arrhythmias - additional genes v3.5 TANGO2 Achchuthan Shanmugasundram Publications for gene: TANGO2 were set to 26805782; 30245509
Cardiac arrhythmias - additional genes v3.4 TANGO2 Achchuthan Shanmugasundram Added comment: Comment on phenotypes: This gene has been associated with relevant phenotypes in both OMIM (MIM #616878, accessed on 05 September 2025) and Gene2Phenotype (TANGO2-related infancy-onset recurrent metabolic crises with encephalocardiomyopathy with 'definitive' rating on the DD panel).
Cardiac arrhythmias - additional genes v3.4 TANGO2 Achchuthan Shanmugasundram Phenotypes for gene: TANGO2 were changed from Metabolic encephalomyopathic crises, recurrent, with rhabdomyolysis, cardiac arrhythmias, and neurodegeneration 616878 to Metabolic encephalomyopathic crises, recurrent, with rhabdomyolysis, cardiac arrhythmias, and neurodegeneration, OMIM:616878; recurrent metabolic encephalomyopathic crises-rhabdomyolysis-cardiac arrhythmia-intellectual disability syndrome, MONDO:0018820
Cardiac arrhythmias - additional genes v3.3 TANGO2 Achchuthan Shanmugasundram reviewed gene: TANGO2: Rating: GREEN; Mode of pathogenicity: None; Publications: 26805781, 26805782, 30245509, 31339582, 32929747; Phenotypes: Metabolic encephalomyopathic crises, recurrent, with rhabdomyolysis, cardiac arrhythmias, and neurodegeneration, OMIM:616878, recurrent metabolic encephalomyopathic crises-rhabdomyolysis-cardiac arrhythmia-intellectual disability syndrome, MONDO:0018820; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Cardiac arrhythmias - additional genes v1.15 TANGO2 Ivone Leong Tag for-review was removed from gene: TANGO2.
Cardiac arrhythmias - additional genes v1.15 TANGO2 Ivone Leong commented on gene: TANGO2
Cardiac arrhythmias - additional genes v1.11 TANGO2 Arina Puzriakova Classified gene: TANGO2 as Amber List (moderate evidence)
Cardiac arrhythmias - additional genes v1.11 TANGO2 Arina Puzriakova Added comment: Comment on list classification: Changed rating from Green to Amber so that Green genes on this panel reflect the NHS signed-off version. This will be reviewed at the next GMS panel update (added 'for-review' tag).
Cardiac arrhythmias - additional genes v1.11 TANGO2 Arina Puzriakova Gene: tango2 has been classified as Amber List (Moderate Evidence).
Cardiac arrhythmias - additional genes v1.10 TANGO2 Arina Puzriakova Tag for-review tag was added to gene: TANGO2.
Cardiac arrhythmias - additional genes v1.10 TANGO2 Sarah Leigh Classified gene: TANGO2 as Green List (high evidence)
Cardiac arrhythmias - additional genes v1.10 TANGO2 Sarah Leigh Gene: tango2 has been classified as Green List (High Evidence).
Cardiac arrhythmias - additional genes v1.9 TANGO2 Sarah Leigh gene: TANGO2 was added
gene: TANGO2 was added to Cardiac arrhythmias - additional genes. Sources: Expert Review
Mode of inheritance for gene: TANGO2 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: TANGO2 were set to 26805782; 30245509
Phenotypes for gene: TANGO2 were set to Metabolic encephalomyopathic crises, recurrent, with rhabdomyolysis, cardiac arrhythmias, and neurodegeneration 616878
Review for gene: TANGO2 was set to GREEN
Added comment: Associated with relevant phenotype in OMIM and as confirmed Gen2Phen gene. At least 10 variants reported in at least 14 unrelated cases, with arrhythmias in 8 unrelated cases.

Gene suggested by Rowenna Roberts, Clinical Scientist.
Sources: Expert Review