Sandhoff(Roger),Hepbildikler(Stefan T),Jen... J Biol Chem 2002-07-12

...Hexb-/-), both beta-hexosaminidase alpha and beta-subunits (Hexa-/- and Hexb-/-), GD3 synthase (GD3S-/-), GD3 synthase a...

Jeyakumar(Mylvaganam),Smith(David),Eliott-... Neurobiol Dis 2002-11-15

...Hexb-/-) mouse has severe neurological disease and mimics the human infantile onset variant. However, the Tay-Sachs (Hex...

Jeyakumar(M),Norflus(F),Tifft(C J),Cortina... Blood 2001-02-15

...HEXB gene, causing a deficiency in beta-hexosaminidase. Bone marrow transplantation (BMT), which augments enzyme levels,...

Gomez-Lira(M),Mottes(M),Perusi(C),Pignatti... Mol Cell Probes 2001-07-05

...HEXB mRNA levels.

Noensie(E N),Dietz(H C) Nat Biotechnol 2001-08-09

...HEXB) genes, respectively. A list of genes was produced in which the MLH1 and HEXB genes were among the top 1% of candid...

Cordeiro(P),Hechtman(P),Kaplan(F) Genet Med 2001-11-01

...HEXB (Sandhoff disease and variants), and GM2A (AB variant of GM2 gangliosidosis). We report the establishment of three ...

Schuette(C G),Weisgerber(J),Sandhoff(K) Glycobiology 2001-10-25

...HEXB gene lead to Sandhoff disease, a glycolipid storage disorder characterized by severe neurodegeneration. So far, lit...

Tsukamoto(T),Fukami(H),Yamanaka(S),Yamaguc... Jpn J Cancer Res 2001-05-17

...Hexb, respectively), real-time relative quantitative reverse transcription-polymerase chain reaction (RT-PCR) analysis w...

Teixeira(C A),Sena-Esteves(M),Lopes(L),Sá ... Hum Gene Ther 2001-12-04

...HexB (beta beta dimer) depletion corresponding to about 10% of control HexB activity. The newly synthesized enzyme is co...

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...