Gene Disease Score gda Association Type Original DB Sentence supporting the association PMID PMID Year
Entrez Id: 3674
Gene Symbol: ITGA2B
ITGA2B
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 Biomarker BEFREE The relative synonymous codon usage analysis revealed variation in codon usage and two synonymous codons namely ATA (Ile) and GTA (Val) were absent in NKX-2.5 gene across selected mammalian species suggesting that these two codons were possibly selected against during evolution. 31377427

2020

Entrez Id: 6442
Gene Symbol: SGCA
SGCA
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 GeneticVariation BEFREE We created a 51-point ATFS, that consisted of three ambulation items adapted for ataxia telangiectasia in the frame of FMS (home, school, outdoors), five ADL items, and one schooling item. 31468510

2020

Entrez Id: 5888
Gene Symbol: RAD51
RAD51
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 Biomarker BEFREE These have resulted in higher resolution structural models of the signalling proteins ATM (ataxia telangiectasia mutated), and ATR (ataxia telangiectasia and Rad3-related protein), along with various structures of Rad51. 31748913

2020

Entrez Id: 1436
Gene Symbol: CSF1R
CSF1R
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 Biomarker BEFREE We created a 51-point ATFS, that consisted of three ambulation items adapted for ataxia telangiectasia in the frame of FMS (home, school, outdoors), five ADL items, and one schooling item. 31468510

2020

Entrez Id: 4780
Gene Symbol: NFE2L2
NFE2L2
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 AlteredExpression BEFREE Activation of NRF2 by dexamethasone in ataxia telangiectasia cells involves KEAP1 inhibition but not the inhibition of p38. 31107893

2019

Entrez Id: 7422
Gene Symbol: VEGFA
VEGFA
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 GeneticVariation BEFREE Patients with CAS and nonfunctional <i>POT1</i> did not repress ATR (ataxia telangiectasia RAD3-related)-dependent DNA damage signaling and showed a constitutive increase of cell cycle arrest and somatic activating mutations in the VEGF (vascular endothelial growth factor)/angiogenesis pathway (<i>KDR</i> gene). 31510873

2019

Entrez Id: 2551
Gene Symbol: GABPA
GABPA
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 AlteredExpression BEFREE Activation of NRF2 by dexamethasone in ataxia telangiectasia cells involves KEAP1 inhibition but not the inhibition of p38. 31107893

2019

Entrez Id: 738
Gene Symbol: VPS51
VPS51
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 Biomarker BEFREE Tissue transglutaminase (TG2), a potent cross-linking enzyme, is known to be transcriptionally activated by inflammatory cytokines and stabilize angiotensin II (Ang II) receptor AT1 (AT1R) via ubiquitination-preventing posttranslational modification. 30715101

2019

Entrez Id: 285
Gene Symbol: ANGPT2
ANGPT2
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 Biomarker BEFREE Tissue transglutaminase (TG2), a potent cross-linking enzyme, is known to be transcriptionally activated by inflammatory cytokines and stabilize angiotensin II (Ang II) receptor AT1 (AT1R) via ubiquitination-preventing posttranslational modification. 30715101

2019

Entrez Id: 3239
Gene Symbol: HOXD13
HOXD13
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 AlteredExpression BEFREE The serum SPD level was significantly lower (P < .001) in AERD compared with ATA patients, and negatively correlated with fall in FEV<sub>1</sub> (%) after lysine-aspirin bronchoprovocation test and/or the urinary LTE4 level. 29663427

2019

Entrez Id: 26291
Gene Symbol: FGF21
FGF21
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 Biomarker BEFREE We summarise the role of the amino acid transporter B<sup>0</sup>AT1 (SLC6A19) and peptide transporter PEPT1 (SLC15A1) in the modulation of global insulin signalling via the liver-secreted hormone fibroblast growth factor 21 (FGF21). 30936244

2019

Entrez Id: 9851
Gene Symbol: KIAA0753
KIAA0753
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 GeneticVariation BEFREE In addition to these, KIAA0753 variants were recently described in seven patients with Jeune asphyxiating thoracic dystrophy (ATD) (two first cousins, one unrelated patient and one fetus), Joubert syndrome (two siblings) and orofaciodigital syndrome type 6 (one patient). 31816441

2019

Entrez Id: 91607
Gene Symbol: SLFN11
SLFN11
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 AlteredExpression BEFREE The antitumor activity of trabectedin was evaluated under different expression levels of SLFN11 regulated by RNA interference and CRISPR-Cas9 systems, and the combined antitumor activity of ataxia telangiectasia and Rad3-related protein kinase (ATR) inhibitor and trabectedin in sarcoma cell lines using in vitro a cell viability assay and in vivo xenograft models. 31262879

2019

Entrez Id: 256130
Gene Symbol: TMEM196
TMEM196
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 GeneticVariation BEFREE We previously found differences in the minor allele frequency (MAF) of single-nucleotide polymorphisms (SNPs) in transmembrane protein 196 (TMEM196) between 995 patients with aspirin-tolerant asthma (ATA) and 141 asthmatic patients with NSAID-exacerbated respiratory disease (NERD). 30694883

2019

Entrez Id: 100126333
Gene Symbol: MIR708
MIR708
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 AlteredExpression BEFREE In this study, we found that treatment with either the synthetic GC dexamethasone (DEX) or the natural GC mimic, antcin A (ATA) significantly increased miR-708 expression by transactivation of glucocorticoid receptor alpha (GRα) in MCF-7 and MDA-MB-231 human BCCs. 30726934

2019

Entrez Id: 185
Gene Symbol: AGTR1
AGTR1
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 GeneticVariation BEFREE Our in vitro cellular assays showed that LIGHT stimulation triggered a rapid TG2-dependent increase in the abundance of AT1Rs (relative AT1R level after 2-hour LIGHT treatment: AT1R (WT)+TG2 = 2.21 ± 0.23, AT1R (Q315A)+TG2 = 0.18 ± 0.23, P < 0.05 vs. starting point = 1, n = 2) and downstream calcium signaling (fold increase in NFAT-driven luciferase activity: Saline = 0.02 ± 0.03, Ang II = 0.17 ± 0.08, LIGHT = 0.05 ± 0.04, LIGHT+Ang II = 0.90 ± 0.04 (P < 0.01 vs. Ang II), and LIGHT+Ang II+ERW1041E = 0.15 ± 0.15 (P < 0.01 vs. LIGHT+Ang II), n = 3). 30715101

2019

Entrez Id: 6564
Gene Symbol: SLC15A1
SLC15A1
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 Biomarker BEFREE We summarise the role of the amino acid transporter B<sup>0</sup>AT1 (SLC6A19) and peptide transporter PEPT1 (SLC15A1) in the modulation of global insulin signalling via the liver-secreted hormone fibroblast growth factor 21 (FGF21). 30936244

2019

Entrez Id: 29126
Gene Symbol: CD274
CD274
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 Biomarker BEFREE ATC = anaplastic thyroid cancer; DTC = differentiated thyroid cancer; IHC = immunohistochemical; OS = overall survival; PD-1 = programmed cell death-receptor 1; PD-L1 = programmed cell death-ligand 1; PD-L2 = programmed cell death-ligand 2; PTC = papillary thyroid cancer; TNM = tumor-node-metastasis; Treg = regulatory T cell. 30913016

2019

Entrez Id: 1991
Gene Symbol: ELANE
ELANE
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 Biomarker BEFREE We also showed higher oxidative damage to DNA (↑8-OHdG), proteins (↑AGE, ↑AOPP), and lipids (↑4-HNE, ↑8-isop) in both AT and NBS patients. 31611883

2019

Entrez Id: 2175
Gene Symbol: FANCA
FANCA
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 Biomarker BEFREE Fanconi Anemia and Ataxia Telangiectasia in Siblings who Inherited Unique Combinations of Novel FANCA and ATM Null Mutations. 30339652

2019

Entrez Id: 378938
Gene Symbol: MALAT1
MALAT1
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 AlteredExpression BEFREE HBO at 2.5 ATA significantly increased MALAT1 expression in HCAECs and HCAECs-derived exosomes. 30301563

2019

Entrez Id: 8743
Gene Symbol: TNFSF10
TNFSF10
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 Biomarker BEFREE In an orthotopic mouse model of TRAIL-resistant ATC, treatment with MADD siRNA alone reduced tumor growth that, when combined with TRAIL, resulted in significant tumor regressions. 30999276

2019

Entrez Id: 7052
Gene Symbol: TGM2
TGM2
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 GeneticVariation BEFREE Our in vitro cellular assays showed that LIGHT stimulation triggered a rapid TG2-dependent increase in the abundance of AT1Rs (relative AT1R level after 2-hour LIGHT treatment: AT1R (WT)+TG2 = 2.21 ± 0.23, AT1R (Q315A)+TG2 = 0.18 ± 0.23, P < 0.05 vs. starting point = 1, n = 2) and downstream calcium signaling (fold increase in NFAT-driven luciferase activity: Saline = 0.02 ± 0.03, Ang II = 0.17 ± 0.08, LIGHT = 0.05 ± 0.04, LIGHT+Ang II = 0.90 ± 0.04 (P < 0.01 vs. Ang II), and LIGHT+Ang II+ERW1041E = 0.15 ± 0.15 (P < 0.01 vs. LIGHT+Ang II), n = 3). 30715101

2019

Entrez Id: 57539
Gene Symbol: WDR35
WDR35
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 GeneticVariation BEFREE The mutation of WDR35 may lead to short rib-polydactyly syndrome (SRP), asphyxiating thoracic dystrophy (ATD, Jeune syndrome) and Ellis van Creveld syndrome. 30790652

2019

Entrez Id: 201163
Gene Symbol: FLCN
FLCN
CUI: C0004135
Disease: Ataxia Telangiectasia
Ataxia Telangiectasia
0.010 AlteredExpression BEFREE Serum FLCN levels were significantly higher in AERD group than in ATA and NC groups (all P < 0.001). 30126026

2018