A Novel Nonsense Mutation in

Bβ-chain gene bleeding phenotype functional analysis hypofibrinogenemia novel nonsense mutation protein modelling

Journal

Biomedicines
ISSN: 2227-9059
Titre abrégé: Biomedicines
Pays: Switzerland
ID NLM: 101691304

Informations de publication

Date de publication:
13 Dec 2020
Historique:
received: 27 10 2020
revised: 03 12 2020
accepted: 10 12 2020
entrez: 16 12 2020
pubmed: 17 12 2020
medline: 17 12 2020
Statut: epublish

Résumé

Congenital hypofibrinogenemia is a rare bleeding disorder characterized by a proportional decrease of functional and antigenic fibrinogen levels. Hypofibrinogenemia can be considered the phenotypic expression of heterozygous loss of function mutations occurring within one of the three fibrinogen genes (

Identifiants

pubmed: 33322159
pii: biomedicines8120605
doi: 10.3390/biomedicines8120605
pmc: PMC7763967
pii:
doi:

Types de publication

Journal Article

Langues

eng

Références

Int J Mol Sci. 2017 Dec 29;19(1):
pubmed: 29286337
Blood Cells Mol Dis. 2013 Mar;50(3):177-81
pubmed: 23266225
Int J Mol Sci. 2018 May 29;19(6):
pubmed: 29844251
Ann N Y Acad Sci. 2001;936:185-204
pubmed: 11460474
Haemophilia. 2020 Jan;26(1):25-32
pubmed: 31709664
Haemophilia. 2008 May;14(3):630-3
pubmed: 18393984
Thromb Haemost. 2018 Nov;118(11):1867-1874
pubmed: 30332696
Thromb Res. 2020 Apr;188:1-4
pubmed: 32018167
Subcell Biochem. 2017;82:405-456
pubmed: 28101869
Hum Mol Genet. 2005 Nov 1;14(21):3271-80
pubmed: 16195396
J Clin Pathol. 1996 Dec;49(12):958
pubmed: 9038728
Blood Coagul Fibrinolysis. 2018 Mar;29(2):155-159
pubmed: 29176380
J Thromb Haemost. 2006 Oct;4(10):2115-29
pubmed: 16999847
Eur J Haematol. 2007 May;78(5):410-6
pubmed: 17331136
Blood Rev. 2015 Jan;29(1):17-24
pubmed: 25294122
J Thromb Haemost. 2005 Oct;3(10):2347-9
pubmed: 16194209
Adv Protein Chem. 2005;70:247-99
pubmed: 15837518
Pathology. 2015 Oct;47(6):583-5
pubmed: 26308135
Thromb Res. 2007;121(3):339-45
pubmed: 17582472
J Thromb Haemost. 2006 Jul;4(7):1634-7
pubmed: 16839371
J Thromb Haemost. 2018 Sep;16(9):1887-1890
pubmed: 30076675
Semin Thromb Hemost. 2016 Jun;42(4):455-8
pubmed: 27148845
Nature. 1997 Oct 2;389(6650):455-62
pubmed: 9333233
Haemophilia. 2017 Jul;23(4):583-589
pubmed: 28306188
Nucleic Acids Res. 2018 Jul 2;46(W1):W296-W303
pubmed: 29788355
Thromb Haemost. 2002 Sep;88(3):427-31
pubmed: 12353071
Thromb J. 2017 Sep 12;15:24
pubmed: 28912669
Arterioscler Thromb Vasc Biol. 2011 Mar;31(3):494-9
pubmed: 21325671
Blood Coagul Fibrinolysis. 2015 Dec;26(8):978-80
pubmed: 26484644
Int J Mol Sci. 2018 Jan 08;19(1):
pubmed: 29316703
J Thromb Haemost. 2007 Jul;5 Suppl 1:125-31
pubmed: 17635718

Auteurs

Tomas Simurda (T)

National Centre of Hemostasis and Thrombosis, Department of Hematology and Transfusiology Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin and University Hospital in Martin, 03601 Martin, Slovakia.

Rui Vilar (R)

Department of Genetic Medicine and Development, University Medical School of Geneva, 1211 Geneva, Switzerland.

Jana Zolkova (J)

National Centre of Hemostasis and Thrombosis, Department of Hematology and Transfusiology Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin and University Hospital in Martin, 03601 Martin, Slovakia.

Eliska Ceznerova (E)

Institute of Hematology and Blood Transfusion, 128 20 Prague, Czech Republic.

Zuzana Kolkova (Z)

Biomedical Center Martin, Jessenius Faculty of Medicine in Martin, Comenius University in Bratislava, 03601 Martin, Slovakia.

Dusan Loderer (D)

Biomedical Center Martin, Jessenius Faculty of Medicine in Martin, Comenius University in Bratislava, 03601 Martin, Slovakia.

Marguerite Neerman-Arbez (M)

Department of Genetic Medicine and Development, University Medical School of Geneva, 1211 Geneva, Switzerland.

Alessandro Casini (A)

Division of Angiology and Hemostasis, University Hospitals of Geneva and Faculty of Medicine, 1205 Geneva, Switzerland.

Monika Brunclikova (M)

National Centre of Hemostasis and Thrombosis, Department of Hematology and Transfusiology Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin and University Hospital in Martin, 03601 Martin, Slovakia.

Ingrid Skornova (I)

National Centre of Hemostasis and Thrombosis, Department of Hematology and Transfusiology Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin and University Hospital in Martin, 03601 Martin, Slovakia.

Miroslava Dobrotova (M)

National Centre of Hemostasis and Thrombosis, Department of Hematology and Transfusiology Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin and University Hospital in Martin, 03601 Martin, Slovakia.

Marian Grendar (M)

Biomedical Center Martin, Jessenius Faculty of Medicine in Martin, Comenius University in Bratislava, 03601 Martin, Slovakia.

Jan Stasko (J)

National Centre of Hemostasis and Thrombosis, Department of Hematology and Transfusiology Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin and University Hospital in Martin, 03601 Martin, Slovakia.

Peter Kubisz (P)

National Centre of Hemostasis and Thrombosis, Department of Hematology and Transfusiology Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin and University Hospital in Martin, 03601 Martin, Slovakia.

Classifications MeSH