Linker proteins restore basement membrane and correct LAMA2-related muscular dystrophy in mice
JR Reinhard, S Lin, KK McKee, S Meinen… - Science translational …, 2017 - science.org
L AMA2-related muscular dystrophy (LAMA2 MD or MDC1A) is the most frequent form of
early-onset, fatal congenital muscular dystrophies. It is caused by mutations in LAMA2, the …
early-onset, fatal congenital muscular dystrophies. It is caused by mutations in LAMA2, the …
Chimeric protein repair of laminin polymerization ameliorates muscular dystrophy phenotype
KK McKee, SC Crosson, S Meinen… - The Journal of …, 2017 - Am Soc Clin Investig
Mutations in laminin α2-subunit (Lmα2, encoded by LAMA2) are linked to approximately
30% of congenital muscular dystrophy cases. Mice with a homozygous mutation in Lama2 …
30% of congenital muscular dystrophy cases. Mice with a homozygous mutation in Lama2 …
Laminin-deficient muscular dystrophy: Molecular pathogenesis and structural repair strategies
PD Yurchenco, KK McKee, JR Reinhard, MA Rüegg - Matrix Biology, 2018 - Elsevier
Laminins are large heterotrimers composed of the α, β and γ subunits with distinct tissue-
specific and developmentally regulated expression patterns. The laminin-α2 subunit …
specific and developmentally regulated expression patterns. The laminin-α2 subunit …
[HTML][HTML] Amelioration of muscle and nerve pathology of Lama2-related dystrophy by AAV9-laminin-αLN linker protein
KK McKee, PD Yurchenco - JCI insight, 2022 - ncbi.nlm.nih.gov
LAMA2 deficiency, resulting from a defective or absent laminin α2 subunit, is a common
cause of congenital muscular dystrophy. It is characterized by muscle weakness from …
cause of congenital muscular dystrophy. It is characterized by muscle weakness from …
Laminin α1 reduces muscular dystrophy in dy2J mice
KI Gawlik, VM Harandi, RY Cheong, Å Petersén… - Matrix Biology, 2018 - Elsevier
Muscular dystrophies, including laminin α2 chain-deficient muscular dystrophy (LAMA2-
CMD), are associated with immense personal, social and economic burdens. Thus, effective …
CMD), are associated with immense personal, social and economic burdens. Thus, effective …
Laminin and integrin in LAMA2-related congenital muscular dystrophy: from disease to therapeutics
P Barraza-Flores, CR Bates… - Frontiers in molecular …, 2020 - frontiersin.org
Laminin-α2-related congenital muscular dystrophy (LAMA2-CMD) is a devastating
neuromuscular disease caused by mutations in the LAMA2 gene. These mutations result in …
neuromuscular disease caused by mutations in the LAMA2 gene. These mutations result in …
Linker protein repair of LAMA2 dystrophic neuromuscular basement membranes
PD Yurchenco, KK McKee - Frontiers in Molecular Neuroscience, 2019 - frontiersin.org
An understanding of basement membrane (BM) assembly at a molecular level provides a
foundation with which to develop repair strategies for diseases with defects of BM structure …
foundation with which to develop repair strategies for diseases with defects of BM structure …
A family of laminin α2 chain-deficient mouse mutants: advancing the research on LAMA2-CMD
KI Gawlik, M Durbeej - Frontiers in Molecular Neuroscience, 2020 - frontiersin.org
The research on laminin α2 chain-deficient congenital muscular dystrophy (LAMA2-CMD)
advanced rapidly in the last few decades, largely due to availability of good mouse models …
advanced rapidly in the last few decades, largely due to availability of good mouse models …
The laminin α2 expressed by dystrophic dy2J mice is defective in its ability to form polymers
H Colognato, PD Yurchenco - Current biology, 1999 - cell.com
Mutations in LAMA2 cause severe congenital muscular dystrophy accompanied by nervous
system defects [1]. Mice homozygous for the dy 2J allele of LAMA2 express a laminin α2 …
system defects [1]. Mice homozygous for the dy 2J allele of LAMA2 express a laminin α2 …
A single point mutation in the LN domain of LAMA2 causes muscular dystrophy and peripheral amyelination
BL Patton, B Wang, YS Tarumi… - Journal of cell …, 2008 - journals.biologists.com
Mutations in the gene encoding the basal lamina (BL) component laminin α2 (LAMA2)
cause merosin-deficient congenital muscular dystrophy 1A (MDC1A), a complex disorder …
cause merosin-deficient congenital muscular dystrophy 1A (MDC1A), a complex disorder …
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