Elevated levels of cystatin C and tenascin-C in schisis cavities of patients with congenital X-linked retinoschisis.
OBJECTIVE
To describe the finding of tenascin C and cystatin-C in the intraschisis cavities of pediatric patients with intraretinal schisis cavities.


METHODS
Three patients with congenital X-linked retinoschisis (CXLRS) and one girl with clinical retinoschisis underwent vitrectomy for vision-threatening schisis cavities. At the time of surgery undiluted samples of intraschisis fluid and vitreous fluid from four eyes (three male and one female) were obtained and analyzed by gel electrophoresis and protein sequencing for the presence of tenascin C and cystatin-C.


RESULTS
Tenascin C and cystatin-C were found in all four samples of fluid from the intraschisis cavities, including a girl with a clinical presentation of CXLRS. The vitreous samples did not have detectable levels of either protein as determined by gel electrophoresis.


CONCLUSIONS
Tenascin C and cystatin-C levels are elevated in intraschisis cavity fluid. Interestingly, this was also found in a girl not carrying a mutation in the retinoschisin gene, indicating that elevated concentrations of tenascin C and cystatin-C result from pathologic changes in the retina and not from the presence of aberrant retinoschisin.
Drenser
Kimberly A
KA
Associated Retinal Consultants, William Beaumont Hospital, Royal Oak, Michigan 48073, USA. kimber@pol.net
Trese
Michael T
MT
Capone
Antonio
A
Hartzer
Michael
M
Dailey
Wendelin
W
D001826:Body Fluids / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D002851:Chromatography, High Pressure Liquid; D055316:Cystatin C; D015891:Cystatins / Q000378:metabolism*; D004591:Electrophoresis, Polyacrylamide Gel; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D041441:Retinoschisis / Q000151:congenital / Q000378:metabolism* / Q000601:surgery; D019063:Tenascin / Q000378:metabolism*; D014822:Vitreous Body / Q000378:metabolism
C525030:CST3 protein, human; D055316:Cystatin C; D015891:Cystatins; D019063:Tenascin