Morphological abnormalities in mitochondria of the skin of patients with sporadic amyotrophic lateral sclerosis.
OBJECTIVE
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.
Neurology Department, Hospital Ramos Mejía, Buenos Aires, Argentina. gerodrig@intramed.net
Gabriel E
GE
Rodríguez
Deniselle M Claudia
DM
González
Gisella M Gargiulo
GM
Monachelli
Juan J López
JJ
Costa
Alejandro F de
AF
Nicola
Roberto E P
RE
Sica
D000328:Adult; D000690:Amyotrophic Lateral Sclerosis / Q000473:pathology*; D016022:Case-Control Studies; D006801:Humans; D015603:Keratinocytes / Q000000981:diagnostic imaging*; D008854:Microscopy, Electron; D008875:Middle Aged; D008928:Mitochondria / Q000648:ultrastructure*; D012867:Skin / Q000473:pathology / Q000648:ultrastructure*; D014463:Ultrasonography