Axonal Regeneration and Neuronal Function Are Preserved in Motor Neurons Lacking ß-Actin In Vivo

The proper localization of ß-actin mRNA and protein is essential for growth cone guidance and axon elongation in cultured neurons. In addition, decreased levels of ß-actin mRNA and protein have been identified in the growth cones of motor neurons cultured from a mouse model of Spinal Muscular Atrophy (SMA), suggesting that ß-actin loss-of-function at growth cones or pre-synaptic nerve terminals could contribute to the pathogenesis of this disease. However, the role of ß-actin in motor neurons in vivo and its potential relevance to disease has yet to be examined. We therefore generated motor neuron specific ß-actin knock-out mice (Actb-MNsKO) to investigate the function of ß-actin in motor neurons in vivo. Surprisingly, ß-actin was not required for motor neuron viability or neuromuscular junction maintenance. Skeletal muscle from Actb-MNsKO mice showed no histological indication of denervation and did not significantly differ from controls in several measurements of physiologic function. Finally, motor axon regeneration was unimpaired in Actb-MNsKO mice, suggesting that ß-actin is not required for motor neuron function or regeneration in vivo

Unusual occurrence of cervical myelopathy in a case of Stickler's syndrome.

We report the occurrence of progressive Brown-Séquard syndrome as the presenting clinical feature of cervical spondylosis in a young patient with Stickler's syndrome

Rachitismes. Classification et traitements

SCOPUS: ar.jinfo:eu-repo/semantics/publishe

Synthesis of a Phenylazo-derivative of a 1,2,3-thiadiazolium Methylide - C-13/n-15 Nmr Characterization and Crystal-structure Analysis

The N-phenylhydrazone derived from 4-methoxycarbonyl-1,2,3-thiadiazole-5-carbaldehyde 6 is methylated at the N-3 position, yielding the mesoionic compound 8. The C-13 and N-15 nmr data, as well as the results from a crystal structure analysis, indicate that the molecule is best represented by the resonance forms 8A and 8B. Comparison is made with the previously synthesized thiadiazole derivative 2 which possesses thiapentalene characteristics

2 Triterpenoid Saponins From Heinsia-crinata

Two new triterpenoid saponins isolated from the root bark of Heinsia crinata were characterized as heinsiagenin A-3 beta-O-(beta-D-glucopyranosyl-(1 --> 2)-beta-D-glucopyranosyl-(1 --> 6)-[alpha-L-rhamnopyranosyl-(1 --> 2)]-beta-D-glucopyranosyl-(1 --> 2)-beta-D-glucopyranoside) and heinsiagenin A-3 beta-O-(alpha-L-rhamnopyranosyl-(1 --> 2)-beta-D-glucopyranosyl(1 --> 2)-beta-D-glucopyranoside). The structures were deduced mainly from a combination of 1- and 2D NMR experiments