Asb2β is essential for embryonic cardiomyocyte development and filamin B degradation in epicardium and endocardium in mice

2019 ◽  
Vol 88 ◽  
pp. 23-24
Author(s):  
Seul Gi Park ◽  
Eun-Kyoung Kim ◽  
Ki-Hoan Nam ◽  
Beom Jun Lee ◽  
Young Won Yun ◽  
...  
Keyword(s):  
Filamin B

scholarly journals The filamin-B–refilin axis – spatiotemporal regulators of the actin-cytoskeleton in development and disease

2018 ◽  
Vol 131 (8) ◽  
pp. jcs213959 ◽  
Author(s):  
Jacques Baudier ◽  
Zandra A. Jenkins ◽  
Stephen P. Robertson
Keyword(s):  
Actin Cytoskeleton ◽  
Filamin B

A Case of Boomerang Dysplasia with a Novel Causative Mutation in Filamin B: Identification of Typical Imaging Findings on Ultrasonography and 3D-CT Imaging

2012 ◽  
Vol 32 (3) ◽  
pp. 216-220
Author(s):  
Seiji Tsutsumi ◽  
Ayako Maekawa ◽  
Miyuki Obata ◽  
Timothy Morgan ◽  
Stephen P. Robertson ◽  
...  
Keyword(s):  
Ct Imaging ◽  
Causative Mutation ◽  
3D Ct ◽  
Imaging Findings ◽  
Filamin B ◽  
3D Ct Imaging

scholarly journals Formin 1 and Filamin B physically interact to coordinate chondrocyte proliferation and differentiation in the growth plate

2014 ◽  
Vol 23 (17) ◽  
pp. 4663-4673 ◽  
Author(s):  
J. Hu ◽  
J. Lu ◽  
G. Lian ◽  
R. J. Ferland ◽  
M. Dettenhofer ◽  
...  
Keyword(s):  
Growth Plate ◽  
Chondrocyte Proliferation ◽  
Proliferation And Differentiation ◽  
Filamin B

scholarly journals Filamin B Mediates ICAM-1-driven Leukocyte Transendothelial Migration

2008 ◽  
Vol 283 (46) ◽  
pp. 31830-31839 ◽  
Author(s):  
Edwin Kanters ◽  
Jos van Rijssel ◽  
Paul J. Hensbergen ◽  
David Hondius ◽  
Frederik P. J. Mul ◽  
...  
Keyword(s):  
Transendothelial Migration ◽  
Leukocyte Transendothelial Migration ◽  
Filamin B

scholarly journals Hypoxia-induced expression of VE-cadherin and filamin B in glioma cell cultures and pseudopalisade structures

2013 ◽  
Vol 113 (2) ◽  
pp. 239-249 ◽  
Author(s):  
Marie-France Nissou ◽  
Michèle El Atifi ◽  
Audrey Guttin ◽  
Catherine Godfraind ◽  
Caroline Salon ◽  
...  
Keyword(s):  
Cell Cultures ◽  
Glioma Cell ◽  
Induced Expression ◽  
Filamin B

scholarly journals Disease-associated mutations in the actin-binding domain of filamin B cause cytoplasmic focal accumulations correlating with disease severity

2012 ◽  
Vol 33 (4) ◽  
pp. 665-673 ◽  
Author(s):  
Philip B. Daniel ◽  
Tim Morgan ◽  
Yasemin Alanay ◽  
Emilia Bijlsma ◽  
Tae-Joon Cho ◽  
...  
Keyword(s):  
Disease Severity ◽  
Binding Domain ◽  
Actin Binding ◽  
Actin Binding Domain ◽  
Filamin B

scholarly journals Implicaciones anestésicas en el Síndrome de Larsen: A propósito de un caso

2019 ◽  
Vol 11 (10) ◽  
pp. 1
Author(s):  
Irene Jarana Aparicio ◽  
Maria Dolores Díaz Lara ◽  
JL Bonilla García
Keyword(s):  
Literature Review ◽  
Anesthetic Management ◽  
Hereditary Disease ◽  
Cytoskeletal Protein ◽  
Anatomical Features ◽  
Live Births ◽  
Larsen Syndrome ◽  
Filamin B ◽  
Genes Encoding

El síndrome de Larsen (SL) es una enfermedad hereditaria rara caracterizada por un defecto en la formación de colágeno debido a mutaciones en los genes que codifican la proteína citoesquelética filamina B. Su prevalencia en Europa es aproximadamente de 1/250.000 nacidos vivos. Esto implica una serie de rasgos y particularidades anatómicas de la vía aérea que debemos valorar en niños que van a ser sometidos a un acto anestésico. Se presenta el caso de un niño de 11 años diagnosticado de síndrome de Larsen que se interviene de aticotomía oído izquierdo. A este propósito, realizamos revisión bibliográfica sobre las peculiaridades del manejo anestésico de estos pacientes. ABSTRACT Anaesthetic implications in Larsen Syndrome: about a case Larsen Syndrome (SL) is a rare hereditary disease characterized by a defect in the formation of collagen due to mutations in the genes encoding the cytoskeletal protein filamin B. Its prevalence in Europe is approximately 1 to 250,000 live births. This implies a number of anatomical features of the airway that we must assess in children who are going under anesthesia. We present the case of an 11-year-old boy diagnosed with Larsen syndrome who underwent left ear aticotomy. In this regard, we conducted a literature review on the peculiarities of anesthetic management of these patients.  

Filaminopathy type C

2021 ◽  
pp. 62-65
Author(s):  
S. V. Kopishinskaia ◽  
A. A. Lesnikova ◽  
D. I. Abramova ◽  
I. A. Velichko
Keyword(s):  
Three Dimensional ◽  
Actin Binding ◽  
Phenotypic Correlation ◽  
Allelic Series ◽  
Actin Networks ◽  
Homologous Genes ◽  
Filamin B ◽  
Cross Links ◽  
Type C ◽  
The Moment

Filamin is an actin-binding protein that, by forming flexible molecular cross-links, stabilizes the three-dimensional F-actin networks and gives them the mechanical properties of a gel. It is represented by three isoforms: filamine A (FLNA), filamin B (FLNB), and filamin C (FLNC), derived from 3 homologous genes. Laminopathies caused by mutations in the FLNA, FLNB, and FLNC genes represent an extensive allelic series of diseases. The review discusses in detail the genotype-phenotypic correlation of all types of phylaminopathies. The neuromuscular and cardiac clinic of C-type phylaminopathy is described in detail. Three variants of C phylaminopathy known at the moment are analyzed.

scholarly journals Myxoma viral serpin, Serp-1, inhibits human monocyte adhesion through regulation of actin-binding protein filamin B

2008 ◽  
Vol 85 (3) ◽  
pp. 418-426 ◽  
Author(s):  
Kasinath Viswanathan ◽  
Jakob Richardson ◽  
Babajide Togonu-Bickersteth ◽  
Erbin Dai ◽  
Liying Liu ◽  
...  
Keyword(s):  
Binding Protein ◽  
Human Monocyte ◽  
Actin Binding ◽  
Monocyte Adhesion ◽  
Actin Binding Protein ◽  
Filamin B
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