24 Fourteen tri-deca polybrominated diphenyl ethers (PBDEs) were investigated in 35 human 25 milk samples from Birmingham, UK. While none of the hepta-nona BDEs (the main 26 components of the OctaBDE technical mixture) were above the limit of quantitation (LOQ); 27 BDE-47 (average concentration = 3.3 ng g lipid weight (lw)) was quantified in all samples 28 contributing 34-74% to Σtri-hexa BDEs ...

We have generated mouse models of human Tay-Sachs and Sandhoff diseases by targeted disruption of the Hexa (alpha subunit) or Hexb (beta subunit) genes, respectively, encoding lysosomal beta-hexosaminidase A (structure, alpha) and B (structure, beta beta). Both mutant mice accumulate GM2 ganglioside in brain, much more so in Hexb -/- mice, and the latter also accumulate glycolipid GA2. Hexa -/-...

The B3LYP/6-31++G** density functional calculations were used to obtain minimum geometries and interaction energies between the molecular hydrogen and nanostructures of fullerenes, C20 (cage), C20 (bowl), C19Si (bowl, penta), C19Si (bowl, hexa). The H2 molecule is set as adsorbed in the distance of 3Å at vertical position from surface above the pentagonal and hexagonal sites of nanostructures. ...

In this communication, we report the synthesis of several novel hexa-(peri)-hexabenzocoronene (HBC) containing microporous polymers. These polymers exhibit good adsorption properties for H2, N2 and CO2 gases. Based on fluorescence quenching studies, we have shown that the highly conjugated π-surfaces may be accessible by guests in solution.

The title compound, [Mn(C(2)H(5)O)(2)(C(44)H(28)N(4))]PF(6), was synthesized from manganese(III) 2,4-penta-nedionate and 5,10,15,20-tetra-phenyl-calix[4]pyrrole by a hydro-thermal reaction. The Mn(III) atom is located on an inversion centre and the asymmetric unit comprises one half-formula unit. The Mn(III) ion is hexa-coordinated by four N atoms from one 5,10,15,20-tetra-phenyl-calix[4]pyrrol...

There are two independent molecules in the asymmetric unit of the title cyclo-hexa-none derivative, C(14)H(24)OS, in which both cyclo-hexane rings exhibit chair conformations. They are also equatorial to each other, which permits the ethanethiol substituent to be in a syn conformation with the α-H atom of the parent attached cyclo-hexa-none.

In the title compound, C(21)H(28)O(4)·H(2)O, the cyclo-hexa-dienone ring is planar (r.m.s. deviation 0.0186 Å), whereas the two cyclo-hexane rings and the cyclo-hexa-none ring adopt chair conformations. The crystal structure is stabilized by O-H⋯O and C-H⋯O hydrogen bonds.

Tay-Sachs or Sandhoff disease result from mutations in either the evolutionarily related HEXA or HEXB genes encoding respectively, the α- or β-subunits of β-hexosaminidase A (HexA). Of the three Hex isozymes, only HexA can interact with its cofactor, the GM2 activator protein (GM2AP), and hydrolyze GM2 ganglioside. A major impediment to establishing gene or enzyme replacement therapy based on H...

We have constructed a defined acapsular mutant in Pasteurella multocida X-73 (serogroup A:1) by disrupting the hexA gene through the insertion of a tetracycline resistance cassette. The genotype of the hexA::tet(M) strain was confirmed by PCR and Southern hybridization, and the acapsular phenotype of this strain was confirmed by electron microscopy. The hexA::tet(M) strain was attenuated in bot...