Activated sialidase activity in transformed lymphocytes by Epstein-Barr (EB) virus of sialidosis type I (cherry-red spot-myoclonus syndrome).
We report a new Japanese family of sialidosis type I. The sialidase activity was deficient in the lymphocytes of 2 patients (6.8% (sister) and 12.5% (brother) of control mean). However, surprisingly, using the transformed lymphocytes by EB virus, this activity was activated to 51.7% (sister) and 49.5% (brother) of control mean, respectively. Although the mechanism for this activation was not known, we discussed the possible mechanisms for this phenomenon.
Yamamoto
M
M
Department of Neurology, Tokyo Medical and Dental University, Japan.
Yamauchi
T
T
Yamamoto
K
K
Kobayashi
T
T
D000293:Adolescent; D000328:Adult; D004789:Enzyme Activation; D005260:Female; D004854:Herpesvirus 4, Human; D006801:Humans; D008213:Lymphocyte Activation; D008214:Lymphocytes / Q000201:enzymology*; D008297:Male; D008875:Middle Aged; D009207:Myoclonus / Q000188:drug therapy / Q000201:enzymology* / Q000235:genetics; D009439:Neuraminidase / Q000172:deficiency / Q000378:metabolism*; D018696:Neuroprotective Agents / Q000627:therapeutic use; D010889:Piracetam / Q000627:therapeutic use; D013577:Syndrome; D014412:Tumor Virus Infections / Q000188:drug therapy / Q000201:enzymology* / Q000235:genetics
D018696:Neuroprotective Agents; D009439:Neuraminidase; D010889:Piracetam