Kleiman(F E),Ramírez(A O),Dodelson de Krem... Hum Mutat 1998-12-08

While screening for new mutations in the HEXB gene, which encodes the beta-subunit of beta-hexosaminidase, a TG deletion...

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...

Hara(A),Uyama(E),Uchino(M),Shimmoto(M),Uts... J Neurol Sci 1998-08-18

...HEXB gene: the G1514-->A substitution (R505Q) and the A619-->G substitution (1207V). The parents were consanguineous. Hi...

Fernandes(M J),Yew(S),Leclerc(D),Henrissat... J Biol Chem 1997-02-12

...HEXB gene product), hydrolyzes neutral substrates. The alpha subunit (HEXA gene product), hydrolyzes both neutral and ch...

Duwat(P),Cochu(A),Ehrlich(S D),Gruss(A) J Bacteriol 1997-08-07

...hexB, and deoB), cell envelope formation (gerC and dltD), various metabolic pathways (arcD, bglA, gidA, hgrP, metB, and ...

Liu(Y),Hoffmann(A),Grinberg(A),Westphal(H)... Proc Natl Acad Sci U S A 1997-08-27

...Hexb -/-) diseases with vastly different clinical phenotypes. The Hexa -/- mice were asymptomatic whereas the Hexb -/- m...

Redonnet-Vernhet(I),Mahuran(D J),Salvayre(... Biochim Biophys Acta 1997-01-03

The molecular defects in the HEXB gene encoding the common beta-subunit of lysosomal beta-hexosaminidase A (beta-Hex A, ...

Mortier-Barriere(I),Humbert(O),Martin(B),P... Microb Drug Resist 1997-10-01

...HexB that can affect the outcome of recombinants, the different levels at which horizontal genetic exchange can be contr...

Tse(R),Vavougios(G),Hou(Y),Mahuran(D J) Biochemistry 1996-07-29

...HEXB genes. This region includes beta Arg211 (invariant in 15 sequences), which we have previously shown to be an active...