Alexander disease, also known as fibrinoid leukodystrophy, is a rare leukoencephalopathy which occurs due to a mutation in the glial fibrillary acid protein (GFAP) gene. Magnetic resonance imaging (MRI) has proven to be highly sensitive in making the diagnosis. Typical MRI findings, in combination with positive genetic blood analysis, confirm the diagnosis.

Whether microglia and macrophages are beneficial or harmful in many neurological disorders, including demyelinating diseases such as multiple sclerosis and the leukodystrophies, is currently under debate. Answering this question is of special interest in globoid cell leukodystrophy (GLD), a genetic fatal demyelinating disease, because its rapidly progressive demyelination in the nervous system ...

The ecmA (pDd63) and ecmB (pDd56) genes encode extracellular matrix proteins of the slime sheath and stalk tube of Dictyostelium discoideum. Using fusion genes containing the promoter of one or other gene coupled to an immunologically detectable reporter, we previously identified two classes of prestalk cells in the tip of the migrating slug; a central core of pstB cells, which express the ecmB...

Anson's circumnavigation was characterised by his indomitable leadership which enabled men to triumph over medical and environmental disasters responsible for appalling suffering and loss of life. The medical bequest of the Anson voyage was not only the detailed descriptions of nutritional deficiency diseases in their various guises, but in the spirit of inquiry it generated, the recognition of...

Sublethal stress in Escherichia coli was detected in various test media after exposure (in vitro) to seawater of various salinites. Stress was measured with an electrochemical detection technique and a beta-galactosidase assay. Test media included EC medium, medium A-1, and tryptic soy broth modified to contain lactose for beta-galactosidase assay experiments. Stress was defined as the differen...

A counterpart of the antibody-mediated activation of genetically defective enzymes is reported here. Antibodies elicited by certain mutant forms of beta-D-galactosidase (EC 3.2.1.23) of Escherichia coli were found to inactivate the normal form of the enzyme. (Antibodies elicited by normal beta-D-galactosidase do not affect the enzyme's catalytic activity.) We present evidence that the inactivat...

Inhibition kinetics of single-beta-galactosidase molecules with the slow-binding inhibitor d-galactal have been characterized by segregating individual enzyme molecules in an array of 50,000 ultra small reaction containers and observing substrate turnover changes with fluorescence microscopy. Inhibited and active states of beta-galactosidase could be clearly distinguished, and the large array s...

A DNA construct containing the bacterial beta-galactosidase gene (lacZ) was used to study somatic DNA recombination in the transgenic mouse brain. Recombination-positive areas of the adult brain were stained blue with X-gal, a substrate of beta-galactosidase. Blue-colored cells appeared soon after birth, and continued to emerge in postnatal tissue. Staining was prominent in sensory as opposed t...