Buccoliero(R),Ginzburg(L),Futerman(A H) J Inherit Metab Dis 2005-03-24

...Hexb mouse, a model of Sandhoff disease, lipid phosphate levels were elevated in surfactant from 3- and 4-month-old mice...

Hitchman(T S),Schmidt(E W),Trail(F),Rarick... Bioorg Chem 2005-12-14

...HexB, respectively. Comparison of the HexA and HexB sequences with those of the ST FAS subunits and primary metabolic FA...

Tsuji(Daisuke),Kuroki(Aya),Ishibashi(Yasuh... J Neurochem 2005-10-27

...HEXB) associated with simultaneous deficiencies of beta-hexosaminidase A (HexA; alphabeta) and B (HexB; betabeta), and e...

Buccoliero(Rosaria),Bodennec(Jacques),Van ... J Neurochem 2004-08-06

Sandhoff disease is a progressive neurodegenerative disorder caused by mutations in the HEXB gene which encodes for the ...

Martin(Douglas R),Krum(Barbara K),Varadara... Exp Neurol 2004-05-10

...HEXB) coding sequence, which introduces three amino acid substitutions at the carboxyl terminus of the protein and a tra...

Zarghooni(Maryam),Bukovac(Scott),Tropak(Mi... Biochem Biophys Res Commun 2004-12-14

...HEXB gene, while four from the other are removed posttranslationally from the pro-beta-subunit. Natural substrate assays...

Juneja(Subhash C) Reprod Fertil Dev 2004-10-07

...Hexb(-/-) mice. Mating behaviour of Hexb(-/-) mice was assessed at different ages. The ovarian function of Hexb(-/-) fem...

Yamaguchi(Akira),Katsuyama(Kayoko),Suzuki(... J Mol Med (Berl) 2004-03-12

...HEXB gene coding for the beta subunit of the lysosomal hexosaminidases A and B. HEXB mutations result in the accumulatio...

Yamaguchi(Akira),Katsuyama(Kayoko),Nagaham... J Clin Invest 2004-02-09

Mice containing a disruption of the Hexb gene have provided a useful model system for the study of the human lysosomal s...

Mercuri(F),Marinelli(M),Zammit(U),Huang(C ... Phys Rev E Stat Nonlin Soft... 2004-06-02

...HexB (Smectic-A-hexatic-B) in racemic A7 [4-(3-methyl-2-chlorobutanoyloxy)-4(')-heptyloxybiphenyl] and 65OBC (n-hexyl-4(...