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The vasopressin precursor is not processed in the hypothalamus of Wolfram syndrome patients with diabetes insipidus: evidence for the involvement of PC2 and 7B2.

Authors :
Gabreëls BA
Swaab DF
de Kleijn DP
Dean A
Seidah NG
Van de Loo JW
Van de Ven WJ
Martens GJ
Van Leeuwen FW
Source :
The Journal of clinical endocrinology and metabolism [J Clin Endocrinol Metab] 1998 Nov; Vol. 83 (11), pp. 4026-33.
Publication Year :
1998

Abstract

Wolfram syndrome (WS) is characterized by optic atrophy, insulin-dependent diabetes mellitus, vasopressin (VP)-sensitive diabetes insipidus, and neurosensory hearing loss. Here we report a disturbance in VP precursor processing in the supraoptic and paraventricular nuclei of WS patients. In these patients with diabetes insipidus we could hardly detect any cellular immunoreactivity for processed VP in the supraoptic and paraventricular nuclei. On the other hand, in the paraventricular nucleus a considerable number of cells immunoreactive for the VP precursor were present. In addition, the proprotein convertase PC2 and the molecular chaperone 7B2 were absent. As expression of PC2 and 7B2 was detected in the nearby nucleus basalis of Meynert of one WS patient and in the anterior lobe of the other WS patient, the absence of the two proteins in the paraventricular nucleus was not due to mutations in their genes. These results indicate that in WS patients with diabetes insipidus, not only does VP neuron loss occur in the supraoptic nucleus, but there is also a defect in VP precursor processing.

Details

Language :
English
ISSN :
0021-972X
Volume :
83
Issue :
11
Database :
MEDLINE
Journal :
The Journal of clinical endocrinology and metabolism
Publication Type :
Academic Journal
Accession number :
9814487
Full Text :
https://doi.org/10.1210/jcem.83.11.5158