Gene Score gda Association Type Type Original DB Sentence supporting the association PMID PMID Year
Entrez Id: 5291
Gene Symbol: PIK3CB
PIK3CB
0.010 GeneticVariation disease BEFREE Megalencephaly-capillary malformation syndrome (MCAP) is a brain overgrowth disorder characterized by cortical malformations (specifically polymicrogyria), vascular anomalies, and segmental overgrowth secondary to somatic activating mutations in the PI3K-AKT-MTOR pathway (PIK3CA). 31729162 2020
Entrez Id: 5293
Gene Symbol: PIK3CD
PIK3CD
0.010 GeneticVariation disease BEFREE Megalencephaly-capillary malformation syndrome (MCAP) is a brain overgrowth disorder characterized by cortical malformations (specifically polymicrogyria), vascular anomalies, and segmental overgrowth secondary to somatic activating mutations in the PI3K-AKT-MTOR pathway (PIK3CA). 31729162 2020
Entrez Id: 5294
Gene Symbol: PIK3CG
PIK3CG
0.010 GeneticVariation disease BEFREE Megalencephaly-capillary malformation syndrome (MCAP) is a brain overgrowth disorder characterized by cortical malformations (specifically polymicrogyria), vascular anomalies, and segmental overgrowth secondary to somatic activating mutations in the PI3K-AKT-MTOR pathway (PIK3CA). 31729162 2020
Entrez Id: 55388
Gene Symbol: MCM10
MCM10
0.010 Biomarker disease BEFREE Recently, the O'Donnell laboratory has been better able to map the interactions of Mcm10 with a larger Cdc45/GINS/MCM (CMG) unwinding complex placing it at the front of the replication fork. 31014174 2019
Entrez Id: 8318
Gene Symbol: CDC45
CDC45
0.010 Biomarker disease BEFREE <b>Abbreviations:</b> alt-NHEJ: Alternative Nonhomologous End-Joining; CC: Coli-Coil motif; CMG: Cdc45/GINS/MCM2-7; CMGM: Cdc45/GINS/Mcm2-7/Mcm10; CPT: Camptothecin; CSB: Cockayne Syndrome Group B protein; CTD: C-Terminal Domain; DSB: Double-Strand Break; DSBR: Double-Strand Break Repair; dsDNA: Double-Stranded DNA; GINS: <i>go-ichi-ni-</i>san, Sld5-Psf1-Psf2-Psf3; HJ Dis: Holliday Junction dissolution; HJ Res: Holliday Junction resolution; HR: Homologous Recombination; ICL: Interstrand Cross-Link; ID: Internal Domain; MCM: Minichromosomal Maintenance; ND: Not Determined; NTD: N-Terminal Domain; PCNA: Proliferating Cell Nuclear Antigen; RPA: Replication Protein A; SA: Strand Annealing; SE: Strand Exchange; SEW: Steric Exclusion and Wrapping; ssDNA: Single-Stranded DNA; TCR: Transcription-Coupled Repair; TOP1: Topoisomerase. 31014174 2019
Entrez Id: 23476
Gene Symbol: BRD4
BRD4
0.010 Biomarker disease BEFREE Cell samples (i.e., primary fibroblasts) were derived from cultured tissues obtained from six PROS patients [3 boys, 3 girls; aged 2 to 17 years] whose spectrum of PIK3A-related overgrowth included HHML [hemihyperplasia multiple lipomatosis; n = 1], CLOVES [congenital lipomatosis, overgrowth, vascular malformations, epidermal nevi, spinal/skeletal anomalies, scoliosis; n = 1], and MCAP [megalencephaly capillary malformation syndrome; n = 4]. 29549527 2018
Entrez Id: 4763
Gene Symbol: NF1
NF1
0.010 Biomarker disease BEFREE Those initial imaging were reviewed by an experienced neuroradiologist, who confirmed the diagnosis in 34 children (1.3%): 13 had neurofibromatosis-1 (NF-1), 9 had epilepsy, 3 had macrocephaly capillary malformation (MCM) syndrome, 3 had autistic spectrum disorder, 1 had a Chiari-1 malformation, and 1 had increased head circumference. 28406584 2017
Entrez Id: 29126
Gene Symbol: CD274
CD274
0.010 AlteredExpression disease BEFREE Significant differences exist in PD-L1 expression between MCM and MUM. 29185395 2017
Entrez Id: 5781
Gene Symbol: PTPN11
PTPN11
0.010 GeneticVariation disease BEFREE To our knowledge, this report is the first description of a PTPN11 germline variant in an MCAP patient. 24939587 2015
Entrez Id: 1234
Gene Symbol: CCR5
CCR5
0.010 Biomarker disease BEFREE We herein report the impact of MHC haplotype on the outcome of 21 MCM infections with the CCR5-tropic simian/human immunodeficiency virus (SHIV)(SF162P4cy). 22494179 2012
Entrez Id: 5921
Gene Symbol: RASA1
RASA1
0.010 GeneticVariation disease BEFREE There have been significant recent advances in the past several years in the field of neurocutaneous vascular syndromes, including the development of more stringent diagnostic criteria for PHACE syndrome, the renaming of macrocephaly-cutis marmorata telangiectatica congenita to macrocephaly-capillary malformation to accurately reflect the true nature of the syndrome, and discovery of new genetic mutations such as RASA-1. 20582592 2010
Entrez Id: 8831
Gene Symbol: SYNGAP1
SYNGAP1
0.010 GeneticVariation disease BEFREE There have been significant recent advances in the past several years in the field of neurocutaneous vascular syndromes, including the development of more stringent diagnostic criteria for PHACE syndrome, the renaming of macrocephaly-cutis marmorata telangiectatica congenita to macrocephaly-capillary malformation to accurately reflect the true nature of the syndrome, and discovery of new genetic mutations such as RASA-1. 20582592 2010
Entrez Id: 2475
Gene Symbol: MTOR
MTOR
0.020 GeneticVariation disease BEFREE Megalencephaly-capillary malformation syndrome (MCAP) is a brain overgrowth disorder characterized by cortical malformations (specifically polymicrogyria), vascular anomalies, and segmental overgrowth secondary to somatic activating mutations in the PI3K-AKT-MTOR pathway (PIK3CA). 31729162 2020
Entrez Id: 207
Gene Symbol: AKT1
AKT1
0.020 GeneticVariation disease BEFREE Megalencephaly-capillary malformation syndrome (MCAP) is a brain overgrowth disorder characterized by cortical malformations (specifically polymicrogyria), vascular anomalies, and segmental overgrowth secondary to somatic activating mutations in the PI3K-AKT-MTOR pathway (PIK3CA). 31729162 2020
Entrez Id: 4594
Gene Symbol: MMUT
MMUT
0.020 Biomarker disease BEFREE Recently, the O'Donnell laboratory has been better able to map the interactions of Mcm10 with a larger Cdc45/GINS/MCM (CMG) unwinding complex placing it at the front of the replication fork. 31014174 2019
Entrez Id: 4594
Gene Symbol: MMUT
MMUT
0.020 Biomarker disease BEFREE The first involved treatment using Dichlorodimethylsilane MCM 41(TD) and later treatment of a pure sample with sulfuric acid MCM-41. 31850301 2019
Entrez Id: 207
Gene Symbol: AKT1
AKT1
0.020 GeneticVariation disease BEFREE Recent studies have discovered a group of overgrowth syndromes, such as congenital lipomatous overgrowth with vascular, epidermal, and skeletal anomalies (CLOVES) syndrome, Proteus syndrome, and megalencephaly-capillary malformation-polymicrogyria (MCAP) syndrome, are caused by somatic activating variants in genes involved in the phosphatidylinositol 3-kinase/AKT/mechanistic target of rapamycin pathway. 28502725 2017
Entrez Id: 2475
Gene Symbol: MTOR
MTOR
0.020 GeneticVariation disease BEFREE Recent studies have discovered a group of overgrowth syndromes, such as congenital lipomatous overgrowth with vascular, epidermal, and skeletal anomalies (CLOVES) syndrome, Proteus syndrome, and megalencephaly-capillary malformation-polymicrogyria (MCAP) syndrome, are caused by somatic activating variants in genes involved in the phosphatidylinositol 3-kinase/AKT/mechanistic target of rapamycin pathway. 28502725 2017
Entrez Id: 5296
Gene Symbol: PIK3R2
PIK3R2
0.100 CausalMutation disease CLINVAR A combination of genetic and biochemical analyses for the diagnosis of PI3K-AKT-mTOR pathway-associated megalencephaly. 28086757 2017
Entrez Id: 10000
Gene Symbol: AKT3
AKT3
0.100 CausalMutation disease CLINVAR A combination of genetic and biochemical analyses for the diagnosis of PI3K-AKT-mTOR pathway-associated megalencephaly. 28086757 2017
Entrez Id: 10000
Gene Symbol: AKT3
AKT3
0.100 CausalMutation disease CLINVAR Sudden death in a case of megalencephaly capillary malformation associated with a de novo mutation in AKT3. 25416470 2015
Entrez Id: 10000
Gene Symbol: AKT3
AKT3
0.100 CausalMutation disease CLINVAR Germline activating AKT3 mutation associated with megalencephaly, polymicrogyria, epilepsy and hypoglycemia. 25523067 2015
Entrez Id: 5296
Gene Symbol: PIK3R2
PIK3R2
0.100 CausalMutation disease CLINVAR Characterisation of mutations of the phosphoinositide-3-kinase regulatory subunit, PIK3R2, in perisylvian polymicrogyria: a next-generation sequencing study. 26520804 2015
Entrez Id: 10000
Gene Symbol: AKT3
AKT3
0.100 CausalMutation disease CLINVAR AKT3 and PIK3R2 mutations in two patients with megalencephaly-related syndromes: MCAP and MPPH. 23745724 2014
Entrez Id: 5296
Gene Symbol: PIK3R2
PIK3R2
0.100 CausalMutation disease CLINVAR Megalencephaly syndromes: exome pipeline strategies for detecting low-level mosaic mutations. 24497998 2014