Adamali(H I),Somani(I H),Huang(J Q),Mahura... J Androl 2000-01-10

...Hexb -/-) and wild-type (Hexb +/+) mice were perfuse fixed and analyzed by routine light and electron microscopy (LM and...

Furihata(K),Drousiotou(A),Hara(Y),Christop... Hum Mutat 1999-03-11

...HEXB gene encoding the beta-subunit of beta-hexosaminidase. In this study, we analyzed the HEXB gene of a Sandhoff carri...

Bertoni(C),Li(Y T),Li(S C) J Biol Chem 1999-11-02

...HexB to hydrolyze GA2, whereas the human activator is ineffective in stimulating the hydrolysis of GA2 (Yuziuk, J. A., B...

Rosey(E L),Lincoln(R A),Ward(P N),Yancey(R... FEMS Microbiol Lett 1999-10-14

...hexB, and not on a DNA fragment between the genes encoding an ATP binding cassette transporter protein (abc) and a prote...

Mahuran(D J) Biochim Biophys Acta 1999-12-08

...HEXB) subunits of beta-hexosaminidase A, the third is a small glycolipid transport protein, the GM2 activator protein (G...

Sandhoff(K),Kolter(T) Acta Biochim Pol 1999-01-20

...HEXB -/-) turned out to be useful models for known human diseases whereas double knock out mice (HEXA -/- and HEXB -/-) ...

Okamoto(Y),Omichi(K),Yamanaka(S),Ikenaka(K... J Biochem 1999-06-25

...Hexb, and both Hexa and Hexb (double knockout) gene-disrupted mice, but BA-1 was not found in the fractions from Hexb ge...

Jeyakumar(M),Butters(T D),Cortina-Borja(M)... Proc Natl Acad Sci U S A 1999-06-24

...HEXB gene, which encodes the beta-subunit of beta-hexosaminidase. GM2 ganglioside fails to be degraded and accumulates w...

Huang(J Q),Trasler(J M),Igdoura(S),Michaud... Hum Mol Genet 1998-01-02

...Hexb-/- (Sandhoff) mice succumb to a profound neurodegenerative disease by 4-6 months of age. Here we find that neuron d...

Narkis(G),Adam(A),Jaber(L),Pennybacker(M),... Hum Mutat 1998-01-02

...HEXB gene encoding the beta-subunits of Hex that result in heat-labile Hex B thus may lead to erroneous enzymatic genoty...