Using an LEC-induced GSD mouse model, the effect of Sema3A was examined by injecting lentivirus-expressing Sema3A into the tibiae in vivo. We found that the overexpression of Sema3A in tibiae suppressed the expansion of LECs and alleviated bone loss, whereas the injection of lentivirus expressing Sema3A short hairpin RNA (shRNA) into the tibiae ...

To gain further insights into the molecular mechanisms of the disease and to evaluate potential treatment strategies, we have recently developed novel mouse models in which the catalytic subunit of glucose-6 phosphatase (G6pc) is deleted in each glucose-producing organ specifically.

Here we describe the first ROSA26 knock-in mouse for the tissue-specific expression of a Gs-coupled DREADD (rM3Ds-DREADD-green fluorescent protein [GFP], referred to here as “GsD”). This mouse line allows the Cre-dependent expression of Gs-DREADD using tissue-specific Cre drivers or Cre-expressing viruses.

2021年7月6日 · Glycosylation defects in a GSD III mouse model GDE is a key enzyme in glycogenolysis and possesses two independent catalytic activities: oligo-1,4→1,4-glucanotransferase activity transfers 3-glucosyl molecules to the neighboring chain for release by GP, and amylo-1,6-glucosidase activity removes the terminal glucosyl molecule, releasing ...

2004年9月9日 · The use of the GAA-KO/SCID mouse model has verified that the onset of anti-hGAA antibodies may significantly limit the efficacy of therapeutic approaches for GSD-II patients that mount...

032410 C57BL/6N- Gsdmd em4Fcw /J Gsdmd - mice carry a CRISPR/Cas9-derived knock-out allele of the mouse Gsdmd gene. Homozygotes are resistant to LPS-induced septic shock.

2023年6月12日 · We also developed transgenic mice expressing a chemogenetic CD8-restricted Gα s –DREADD to activate CD8-restricted Gα s signaling and show that a Gα s –PKA signaling axis promotes CD8 + T cell...

2024年1月8日 · We found that the overexpression of Sema3A in tibiae suppressed the expansion of LECs and alleviated bone loss, whereas the injection of lentivirus expressing Sema3A short hairpin RNA (shRNA) into...

Glycogen storage diseases (GSDs) of the liver are devastating disorders presenting with fasting hypoglycemia as well as hepatic glycogen and lipid accumulation, which could lead to...

Renal endoplasmic reticulum stress is coupled to impaired autophagy in a mouse model of GSD Ia. by Benjamin L Farah, Dustin J Landau, Yajun Wu, Rohit A Sinha, Alwin Loh, Boon-Huat Bay, Dwight D Koeberl, Paul M Yen. Molecular genetics and metabolism. Read more related scholarly scientific articles and abstracts.