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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.
- 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.
- Subjects :
- Adult
Aged
Aged, 80 and over
Case-Control Studies
Female
Humans
Male
Middle Aged
Molecular Chaperones metabolism
Neuroendocrine Secretory Protein 7B2
Pituitary Hormones metabolism
Proprotein Convertase 2
Protein Precursors metabolism
Subtilisins metabolism
Vasopressins metabolism
Diabetes Insipidus metabolism
Nerve Tissue Proteins metabolism
Paraventricular Hypothalamic Nucleus metabolism
Protein Processing, Post-Translational
Supraoptic Nucleus metabolism
Wolfram Syndrome metabolism
Subjects
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