Kanzaki(Seiichi),Yamaguchi(Akira),Yamaguch... PLoS One 2010-11-04

...Hexb(-/-) mice, an animal model for Sandhoff disease, is associated with the production of pathogenic anti-glycolipid au...

Tamura(S),Tamura(Y),Uchida(K),Nibe(K),Naka... J Vet Intern Med 2010-11-16

...HEXB gene.,To describe the clinical, pathological, biochemical, and magnetic resonance imaging (MRI) findings of Sandhof...

Delnooz(C C S),Lefeber(D J),Langemeijer(S ... J Neurol Neurosurg Psychiat... 2010-09-16

...HEXB gene. Typical, early-onset Sandhoff disease presents before 9 months of age with progressive psychomotor retardatio...

Rutkowska(Justyna),Olszewska(Ewa),Waniewsk... Otolaryngol Pol 2010-04-09

...HEXB mRNA in cholesteatoma. HEXA mRNA in CF was six times higher than in the controls. HEX mRNA expression found to be r...

Matsuoka(Kazuhiko),Tsuji(Daisuke),Aikawa(S... Mol Ther 2010-11-23

Human lysosomal beta-hexosaminidase A is a heterodimer composed of alpha- and beta-subunits encoded by HEXA and HEXB, re...

Denny(Christine A),Heinecke(Karie A),Kim(Y... J Neurochem 2010-08-06

...Hexb) knockout (-/-) murine model of Sandhoff disease. In this study, we used a restricted ketogenic diet (KD-R) and NB-...

Olszewski(Sławomir),Rutkowska(Justyna),Pop... Pol Merkur Lekarski 2010-05-26

...HEXB genes in the synovial cell culture derived from human synovial inflammatory fluid obtained from patients suffered f...

Van Roie(B),Denolf(K),Pitsi(G),Thoen(J) Eur Phys J E Soft Matter 2009-02-19

...HexB) transition in the liquid-crystal n-hexyl-4'-n-pentyloxybiphenyl-4-carboxilate (650BC) with adiabatic scanning calo...

Kyrkanides(Stephanos),Miller(Ann W),Miller... J Neuroimmunol 2009-02-02

...HexB-/- mouse model of Sandhoff disease. Ablation of the chemokine receptor CCR2 in the HexB-/- mouse resulted in signif...

Zampieri(Stefania),Filocamo(Mirella),Burat... Neurogenetics 2009-07-13

...HEXB gene. We identified 11 different mutations of which six are novel: one large deletion of 2,406 nt, (c.299+1471_408d...