Results 61 to 70 of about 32,083 (123)

Effects of Transformer Oil Properties and Contamination on Its Spectral Response

, 2014
UV Spectrophotometry is a non-intrusive test that can be employed to determine power transformer’s integrity. Light transmitted through transformer oil sample containing various contaminations is decreased by that fraction being absorbed and is detected ...
Abu Bakar, Norazhar, Abu-Siada, Ahmed, El-Naggar, Mohamed, Islam, Syed, El-Naggar, M.   +4 more
core  

Disease mechanisms in Lama2-deficiency : a multiorgan approach

Tese de doutoramento em Biologia (Biologia do Desenvolvimento), Universidade de Lisboa, Faculdade de Ciências, 2025.A distrofia muscular congénita relacionada com LAMA2 (LAMA2-CMD) é causada por mutações no gene LAMA2, que codifica a cadeia α2 da ...
Martins ,Susana Gomes Rodrigues
core  

Effects of metformin on congenital muscular dystrophy type 1A disease progression in mice: a gender impact study

, 2018
Congenital muscular dystrophy with laminin α2 chain-deficiency (LAMA2-CMD) is a severe muscle disorder with complex underlying pathogenesis. We have previously employed profiling techniques to elucidate molecular patterns and demonstrated significant ...
M. Soares Oliveira, Bernardo,, Harandi, Vahid,, Durbeej-Hjalt, Madeleine,, Fontes Oliveira, Cibely,, Durbeej-Hjalt, Madeleine, Fontes Oliveira, Cibely, Körner, Zandra,, Körner, Zandra, Harandi, Vahid, Lund University., M. Soares Oliveira, Bernardo   +10 more
core   +1 more source

Electronic structure of fluorides: a DFT-LDA systematic study

, 2008
Electronic structure of fluorides: a DFT-LDA systematic ...
FIORENTINI, VINCENZO, CAPPELLINI, GIANCARLO, Cadelano E   +2 more
core  

Insights Into Early Molecular Events in LAMA2-Congenital Muscular Dystrophy

Tese de mestrado, Biologia Molecular e Genética, 2025, Universidade de Lisboa, Faculdade de CiênciasLAMA2-Congenital Muscular Dystrophy (LAMA2-CMD) is characterized by mutations in the LAMA2 gene, coding for the laminin α2 chain, an important component ...
Alves,Ana Mafalda Laranjo
core  

Effects of N-acetyl-L-cysteine and vitamin E on congenital muscular dystrophy type 1A disease progression in mice

, 2019
Congenital muscular dystrophy with laminin alpha2 chain-deficiency (LAMA2-CMD) is a severe neuromuscular disorder without cure. Previously, we have demonstrated significant metabolic impairment in skeletal muscle from LAMA2-CMD patients and mouse models ...
M. Harandi, Vahid,, Durbeej, Madeleine,, Moreira Soares Oliveira, Bernardo, Durbeej, Madeleine, Moreira Soares Oliveira, Bernardo,, Friberg, Ariana, Gawlik, Kinga,, Gawlik, Kinga, M. Harandi, Vahid, Friberg, Ariana,, Lund University.   +10 more
core   +1 more source

Lama2 lentiviral payload. Directing a treatment for muscular dystrophy

, 2019
Curs 2018-2019In this degree final project will be summarized the results from one of the experimental procedures performed in the Translational Synthetic Biology Department in the PRBB-UPF, a research group which has a project focused on finding a ...
Rivero Dacruz, Andrea
core  

An atypical expression of core α-Dystroglycan and Laminin-α2 in skin fibroblasts of patients with congenital muscular dystrophies. [PDF]

Mol Biol Rep, 2023
Sabry S, Issa MY, Abdel-Hamid MS, Eissa NR, Abdel-Ghafar SF, Ibrahim MM, Zaki MS.   +6 more
europepmc   +1 more source

LAMA2-CMD: establishment of a new gene therapy strategy using an in vitro model

In skeletal muscle tissue, the basement membrane lining the sarcolemma coats and protects myofibers from contraction-induced damage. A key component of this specialized structure is the Laminin-211 (LN211). Pathogenic mutations in the LAMA2 gene, which encodes the α2-chain of laminin-211, trigger a life-threatening and currently incurable disease ...
openaire   +1 more source

Congenital Muscular Dystrophy Due to Merosin Deficiency: Report of a New Mutation. [PDF]

Cureus, 2023
Herrera Malpica WS, Ortiz-Corredor F, Sanchez Peñarete D, Muñetones Hernández PV.   +3 more
europepmc   +1 more source