%214110
CEREBROHEPATORENAL SYNDROME, VARIANT TYPES

Alternative titles; symbols
ZELLWEGER SYNDROME, VARIANT TYPES


TEXT
The complementation studies by somatic cell fusion by Brul et al. (1988) suggested the existence of several forms of Zellweger syndrome (see 214100). Shimozawa et al. (1993) indicated the existence of at least 9 groups of peroxisome-deficient disorders identified by somatic cell fusion studies. For the most part, there was no clear relationship between genotype and phenotype. The phenotype in cases of the same complementation group could be Zellweger syndrome, neonatal adrenoleukodystrophy, or infantile Refsum disease.

REFERENCES
1. Brul, S., Westerveld, A., Strijland, A., Wanders, R. J. A., Schram, A. W., Heymans, H. S. A., Schutgens, R. B. H., van den Bosch, H., Tager, J. M. Genetic heterogeneity in the cerebrohepatorenal (Zellweger) syndrome and other inherited disorders with a generalized impairment of peroxisomal functions: a study using complementation analysis. J. Clin. Invest. 81: 1710-1715, 1988. [PubMed: 2454948, related citations] [Full Text: Journal of Clinical Investigation, Pubget]

2. Shimozawa, N., Suzuki, Y., Orii, T., Moser, A., Moser, H. W., Wanders, R. J. A. Standardization of complementation grouping of peroxisome-deficient disorders and the second Zellweger patient with peroxisomal assembly factor-1 (PAF-1) defect. (Letter) Am. J. Hum. Genet. 52: 843-844, 1993. [PubMed: 7681622, related citations] [Full Text: Pubget]

Creation Date: Victor A. McKusick : 7/11/1989
Edit History: mgross : 03/17/2004
mimadm : 2/19/1994
carol : 5/21/1993
supermim : 3/16/1992
supermim : 3/20/1990
ddp : 10/26/1989
root : 7/11/1989