Morphological abnormalities in mitochondria of the skin of patients with sporadic amyotrophic lateral sclerosis
OBJECTIVES: Mitochondrial dysfunction has been reported in the central nervous system, hepatocytes and peripheral blood lymphocytes from patients with sporadic amyotrophic lateral sclerosis (SALS). However, the status of skin mitochondria has not been reported, in spite of the fact that SALS patient...
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Thieme Revinter Publicações
2012-01-01
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| Series: | Arquivos de Neuro-Psiquiatria |
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| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2012000100010&lng=en&tlng=en |
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| author | Gabriel E. Rodríguez Deniselle M. Claudia González Gisella M. Gargiulo Monachelli Juan J. López Costa Alejandro F. de Nicola Roberto E. P. Sica |
| author_facet | Gabriel E. Rodríguez Deniselle M. Claudia González Gisella M. Gargiulo Monachelli Juan J. López Costa Alejandro F. de Nicola Roberto E. P. Sica |
| author_sort | Gabriel E. Rodríguez |
| collection | DOAJ |
| description | OBJECTIVES: Mitochondrial dysfunction has been reported in the central nervous system, hepatocytes and peripheral blood lymphocytes from patients with sporadic amyotrophic lateral sclerosis (SALS). However, the status of skin mitochondria has not been reported, in spite of the fact that SALS patients present skin abnormalities. The objective of the present study was to compare mitochondrial ultrastructural parameters in keratinocytes from patients with SALS and healthy controls. METHODS: Our study was based on the analysis of 112 skin mitochondria from 5 SALS patients and 99 organelles from 4 control subjects by electron microscopy. RESULTS: Computerized image analysis showed that mitochondrial major axis length, area and perimeter of the organelle were significantly smaller in SALS respect of healthy control subjects. Morphologically, SALS mitochondria presented cristolysis and breakage of the outer membrane. CONCLUSIONS: Mitochondrial dysfunction in the skin may possibly reflect changes occurring in mitochondria of the central nervous system. The analysis of mitochondrial morphology in this tissue may be of value to follow disease progression and, eventually, the effectiveness of current therapies for SALS. |
| format | Article |
| id | doaj-art-a9bd2271ea3347c780c172021f5b7daf |
| institution | OA Journals |
| issn | 1678-4227 |
| language | English |
| publishDate | 2012-01-01 |
| publisher | Thieme Revinter Publicações |
| record_format | Article |
| series | Arquivos de Neuro-Psiquiatria |
| spelling | doaj-art-a9bd2271ea3347c780c172021f5b7daf2025-08-20T02:20:41ZengThieme Revinter PublicaçõesArquivos de Neuro-Psiquiatria1678-42272012-01-01701404410.1590/S0004-282X2012000100010S0004-282X2012000100010Morphological abnormalities in mitochondria of the skin of patients with sporadic amyotrophic lateral sclerosisGabriel E. Rodríguez0Deniselle M. Claudia González1Gisella M. Gargiulo Monachelli2Juan J. López Costa3Alejandro F. de Nicola4Roberto E. P. Sica5Hospital General de Agudos J. M. Ramos MejíaUniversidad de Buenos AiresHospital General de Agudos J. M. Ramos MejíaUniversidad de Buenos AiresUniversidad de Buenos AiresUniversidad de Buenos AiresOBJECTIVES: Mitochondrial dysfunction has been reported in the central nervous system, hepatocytes and peripheral blood lymphocytes from patients with sporadic amyotrophic lateral sclerosis (SALS). However, the status of skin mitochondria has not been reported, in spite of the fact that SALS patients present skin abnormalities. The objective of the present study was to compare mitochondrial ultrastructural parameters in keratinocytes from patients with SALS and healthy controls. METHODS: Our study was based on the analysis of 112 skin mitochondria from 5 SALS patients and 99 organelles from 4 control subjects by electron microscopy. RESULTS: Computerized image analysis showed that mitochondrial major axis length, area and perimeter of the organelle were significantly smaller in SALS respect of healthy control subjects. Morphologically, SALS mitochondria presented cristolysis and breakage of the outer membrane. CONCLUSIONS: Mitochondrial dysfunction in the skin may possibly reflect changes occurring in mitochondria of the central nervous system. The analysis of mitochondrial morphology in this tissue may be of value to follow disease progression and, eventually, the effectiveness of current therapies for SALS.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2012000100010&lng=en&tlng=enesclerosis amiotrófica lateralmicroscopía electrónicaqueratinocitosmitocondria |
| spellingShingle | Gabriel E. Rodríguez Deniselle M. Claudia González Gisella M. Gargiulo Monachelli Juan J. López Costa Alejandro F. de Nicola Roberto E. P. Sica Morphological abnormalities in mitochondria of the skin of patients with sporadic amyotrophic lateral sclerosis Arquivos de Neuro-Psiquiatria esclerosis amiotrófica lateral microscopía electrónica queratinocitos mitocondria |
| title | Morphological abnormalities in mitochondria of the skin of patients with sporadic amyotrophic lateral sclerosis |
| title_full | Morphological abnormalities in mitochondria of the skin of patients with sporadic amyotrophic lateral sclerosis |
| title_fullStr | Morphological abnormalities in mitochondria of the skin of patients with sporadic amyotrophic lateral sclerosis |
| title_full_unstemmed | Morphological abnormalities in mitochondria of the skin of patients with sporadic amyotrophic lateral sclerosis |
| title_short | Morphological abnormalities in mitochondria of the skin of patients with sporadic amyotrophic lateral sclerosis |
| title_sort | morphological abnormalities in mitochondria of the skin of patients with sporadic amyotrophic lateral sclerosis |
| topic | esclerosis amiotrófica lateral microscopía electrónica queratinocitos mitocondria |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2012000100010&lng=en&tlng=en |
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