55 research outputs found
Roughness of Crack Interfaces in Two-Dimensional Beam Lattices
The roughness of crack interfaces is reported in quasistatic fracture, using
an elastic network of beams with random breaking thresholds. For strong
disorders we obtain 0.86(3) for the roughness exponent, a result which is very
different from the minimum energy surface exponent, i.e., the value 2/3. A
cross-over to lower values is observed as the disorder is reduced, the exponent
in these cases being strongly dependent on the disorder.Comment: 9 pages, RevTeX, 3 figure
Friedreich's ataxia associated with mitochondrial myopathy: clinicopathologic report.
A 13-year-old boy with clinical and electrophysiologic findings of Friedreich's ataxia developed unusually prominent myopathy. Skeletal muscle biopsy showed mitochondrial proliferation and structural abnormalities. No mutation was found in skeletal muscle mitochondrial DNA to explain this finding. Molecular genetic and pathologic studies confirmed a diagnosis of Friedreich's ataxia in the proband and affected relatives. Although the Friedreich's ataxia phenotype results from decreased expression of a mitochondrially targeted protein, frataxin, mitochondrial myopathy has not been described as a feature of the disease. The association between the frataxin gene mutation and mitochondrial myopathy in this case suggests that severe or cumulative insults to mitochondrial function may produce myopathic changes in some cases of Friedreich's ataxia. The patient also responded clinically to carnitine supplementation, suggesting a potential palliative therapy for the disease.Case ReportsJournal Articleinfo:eu-repo/semantics/publishe
- …