Terashima(Tomoya),Oka(Kazuhiro),Kritz(Ange... J Clin Invest 2009-07-30

...Hexb) into Hexb-deficient mice, a model of the neuronopathy Sandhoff disease. Delivery of the DRG-targeted HDAd reinstat...

Baek(Rena C),Martin(Douglas R),Cox(Nancy R... Lipids 2009-05-28

...Hexb gene), which catabolizes ganglioside GM2 within lysosomes. Accumulation of GM2 and asialo-GM2 (GA2) occurs primaril...

Gutternigg(Martin),Rendić(Dubravko),Voglau... Biochem J 2009-03-24

...HEXB genes, the third class 20 glycosidase to be identified from mammalian sources.

Kawashima(Nagako),Tsuji(Daisuke),Okuda(Tet... J Neurochem 2009-11-06

...HEXB gene which encodes the beta-subunit of N-acetyl-beta-hexosaminidase A and B, resulting in the accumulation of the g...

Jeyakumar(Mylvaganam),Lee(Jean-Pyo),Sibson... Stem Cells 2009-12-11

...Hexb(-/-)) mice, cells migrated far from the injection site and integrated into the host cytoarchitecture, restoring bet...

Stubbs(Keith A),Macauley(Matthew S),Vocadl... Angew Chem Int Ed Engl 2009-03-23

...HEXB is cell-permeable and modulates the activity of HEXA and HEXB in tissue culture, increasing ganglioside GM2 levels....

Kyrkanides(Stephanos),Yang(Meixiang),Talle... J Neuroimmunol 2009-06-22

...HEXB), encoding for the human HEXB gene, was injected intra-articularly in the temporomandibular joint of 12 week-old He...

Chen(Yanfeng),Allegood(Jeremy),Liu(Ying),W... Anal Chem 2008-05-20

...hexb gene (hexb-/-), a model of Tay-Sachs and Sandhoff disease, versus the functionally normal heterozygote (hexb+/-) by...

Baek(Rena C),Kasperzyk(Julie L),Platt(Fran... Neurochem Int 2008-07-28

...Hexb gene). Accumulation of these glycosphingolipids (GSLs) produces progressive neurodegeneration, ultimately leading t...

Chen(Mei-Hsiu),Liao(Shu-Lang),Chen(Ming-Ho... Invest Ophthalmol Vis Sci 2008-11-18

...HEXB) was reduced as well, but the change did not reach statistical significance (P = 0.058).,Lysosome-related genes, su...