Literature DB >> 32166871

The European Association for Haemophilia and Allied Disorders (EAHAD) Coagulation Factor Variant Databases: Important resources for haemostasis clinicians and researchers.

John H McVey1, Pavithra M Rallapalli2, Geoffrey Kemball-Cook3, Daniel J Hampshire4, Muriel Giansily-Blaizot5, Keith Gomez3, Stephen J Perkins2, Christopher A Ludlam6.   

Abstract

INTRODUCTION: Advances in genomic sequencing have facilitated the sequencing of genes associated with disorders of haemostasis. The identification of variants within genes and access to curated data incorporating structural, functional, evolutionary as well as phenotypic data has become increasingly important in order to ascribe pathogenicity. AIM: The European Association for Haemophilia and Allied Disorders (EAHAD) Coagulation Factor Variant Database Project aims to provide a single port of entry to a web-accessible resource for variants in genes involved in clinical bleeding disorders.
RESULTS: New databases have evolved from previously developed single gene variant coagulation database projects, incorporating new data, new analysis tools and a new common database architecture with new interfaces and filters. These new databases currently present information about the genotype, phenotype (laboratory and clinical) and structural and functional effects of variants described in the genes of factor (F) VII (F7), FVIII (F8), FIX (F9) and von Willebrand factor (VWF).
CONCLUSION: The project has improved the quality and quantity of information available to the haemostasis research and clinical communities, thereby enabling accurate classification of disease severity in order to make assessments of likely pathogenicity.
© 2020 The Authors. Haemophilia published by John Wiley & Sons Ltd.

Entities:  

Keywords:  blood coagulation factors; factor VII; genotype; haemophilia A; haemophilia B; von Willebrand factor

Year:  2020        PMID: 32166871     DOI: 10.1111/hae.13947

Source DB:  PubMed          Journal:  Haemophilia        ISSN: 1351-8216            Impact factor:   4.287


  14 in total

1.  Applicability of the Thrombin Generation Test to Evaluate the Hemostatic Status of Hemophilia A Patients in Daily Clinical Practice.

Authors:  Ángel Bernardo; Alberto Caro; Daniel Martínez-Carballeira; José Ramón Corte; Sonia Vázquez; Carmen Palomo-Antequera; Alfredo Andreu; Álvaro Fernández-Pardo; Julia Oto; Laura Gutiérrez; Inmaculada Soto; Pilar Medina
Journal:  J Clin Med       Date:  2022-06-10       Impact factor: 4.964

Review 2.  Current therapeutic approaches in the management of hemophilia-a consensus view by the Romanian Society of Hematology.

Authors:  Ionut Hotea; Melen Brinza; Cristina Blag; Alina-Andreea Zimta; Noemi Dirzu; Corina Burzo; Ioana Rus; Dragos Apostu; Horea Benea; Mirela Marian; Alexandru Mester; Sergiu Pasca; Sabina Iluta; Patric Teodorescu; Ciprian Jitaru; Mihnea Zdrenghea; Anca Bojan; Tunde Torok-Vistai; Radu Niculescu; Cristina Tarniceriu; Delia Dima; Cristina Truica; Margit Serban; Ciprian Tomuleasa; Daniel Coriu
Journal:  Ann Transl Med       Date:  2021-07

3.  Prediction of hemophilia A severity using a small-input machine-learning framework.

Authors:  Tiago J S Lopes; Ricardo Rios; Tatiane Nogueira; Rodrigo F Mello
Journal:  NPJ Syst Biol Appl       Date:  2021-05-25

Review 4.  Molecular Insights into Determinants of Translational Readthrough and Implications for Nonsense Suppression Approaches.

Authors:  Silvia Lombardi; Maria Francesca Testa; Mirko Pinotti; Alessio Branchini
Journal:  Int J Mol Sci       Date:  2020-12-11       Impact factor: 5.923

5.  Genetic and Bioinformatic Strategies to Improve Diagnosis in Three Inherited Bleeding Disorders in Bogotá, Colombia.

Authors:  Juliana Lago; Helena Groot; Diego Navas; Paula Lago; María Gamboa; Dayana Calderón; Diana C Polanía-Villanueva
Journal:  Genes (Basel)       Date:  2021-11-18       Impact factor: 4.096

6.  Dissection of pleiotropic effects of variants in and adjacent to F8 exon 19 and rescue of mRNA splicing and protein function.

Authors:  Silvia Lombardi; Gabriele Leo; Simone Merlin; Antonia Follenzi; John H McVey; Iva Maestri; Francesco Bernardi; Mirko Pinotti; Dario Balestra
Journal:  Am J Hum Genet       Date:  2021-07-08       Impact factor: 11.025

7.  Molecular determinants of the factor VIII/von Willebrand factor complex revealed by BIVV001 cryo-electron microscopy.

Authors:  James R Fuller; Kevin E Knockenhauer; Nina C Leksa; Robert T Peters; Joseph D Batchelor
Journal:  Blood       Date:  2021-05-27       Impact factor: 22.113

8.  Molecular analysis of 76 Chinese hemophilia B pedigrees and the identification of 10 novel mutations.

Authors:  Limin Huang; Liyan Li; Sheng Lin; Juanjuan Chen; Kun Li; Dongmei Fan; Wangjie Jin; Yihong Li; Xu Yang; Yufeng Xiong; Fenxia Li; Xuexi Yang; Ming Li; Qiang Li
Journal:  Mol Genet Genomic Med       Date:  2020-09-01       Impact factor: 2.183

9.  Novel F8 and F9 gene variants from the PedNet hemophilia registry classified according to ACMG/AMP guidelines.

Authors:  Nadine G Andersson; Veerle Labarque; Anna Letelier; Maria Elisa Mancuso; Martina Bührlen; Kathelijn Fischer; Mutlu Kartal-Kaess; Minna Koskenvuo; Torben Mikkelsen; Rolf Ljung
Journal:  Hum Mutat       Date:  2020-10-14       Impact factor: 4.878

10.  Protein residue network analysis reveals fundamental properties of the human coagulation factor VIII.

Authors:  Tiago J S Lopes; Ricardo Rios; Tatiane Nogueira; Rodrigo F Mello
Journal:  Sci Rep       Date:  2021-06-16       Impact factor: 4.379

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