Transglutaminase activity in serum and cerebrospinal fluid in sporadic amyotrophic lateral sclerosis: a possible use as an indicator of extent of the motor neuron loss.
The activity of transglutaminase (TGase), a marker enzyme for tissue degeneration, was examined in serum and cerebrospinal fluid (CSF) obtained from patients with sporadic amyotrophic lateral sclerosis (SALS), and compared to those from patients without SALS. When the serum TGase activity values from SALS patients were compared against the 'ALS-scale', which is used for clinical evaluation of the progression of ALS, the TGase activity values were higher at the initial stage of the disease than in non-ALS subjects, whereas they became extremely low at the late stage of ALS. The TGase activity in the CSF taken at later than middle stage from ALS patients with definite clinical motor dysfunctions was significantly lower than in that from non-ALS subjects. We have previously demonstrated marked reduction of tissue TGase activity in all regions of spinal cord tissue transections from ALS patients, not only in ventral but also lateral and dorsal regions, relative to that in non-ALS patients. These results suggest that some TGase may be leaked out of the spinal cord tissue into the CSF and then into the blood-stream during the progression of ALS, and the enzymic activity finally becomes depleted at the terminal stages of the disease when most of the spinal motor neuronal perikarya have been destroyed.
Department of Physiology, School of Medicine, Fujita Health University, Aichi, Japan. myamauti@fujita-hu.ac.jp
K
K
Fujita
M
M
Honda
R
R
Hayashi
K
K
Ogawa
M
M
Ando
M
M
Yamauchi
Y
Y
Nagata
D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000097:blood / Q000134:cerebrospinal fluid / Q000201:enzymology* / Q000473:pathology; D015415:Biomarkers; D016923:Cell Death; D002555:Cerebrospinal Fluid / Q000201:enzymology; D002556:Cerebrospinal Fluid Proteins / Q000032:analysis*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000473:pathology*; D009419:Nerve Tissue Proteins / Q000097:blood / Q000134:cerebrospinal fluid*; D013116:Spinal Cord / Q000201:enzymology*; D011503:Transglutaminases / Q000032:analysis* / Q000134:cerebrospinal fluid
D015415:Biomarkers; D002556:Cerebrospinal Fluid Proteins; D009419:Nerve Tissue Proteins; D011503:Transglutaminases