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Localization and distribution of wolframin in human tissues
- Source :
- Scopus-Elsevier
- Publication Year :
- 2012
- Publisher :
- IMR Press, 2012.
-
Abstract
- Wolframin is a transmembrane glycoprotein of 890 aminoacids, encoded by WFS1 gene. WFS1 mutations are responsible for Wolfram syndrome, an autosomal recessive disorder. In the present paper, we first characterized the polyclonal wolframin antibody by dot blot. Secondly, we verified antibody specificity by western blotting using different human cell lines. Thirdly, we studied wolframin localization in human foetal (14-35 weeks) and adult tissues by immunohistochemistry. Wolframin expression was distributed in many organs, with different tissue and cell localization and expression levels. In foetal systems, wolframin expression was faint at 14-16 weeks and increased when development proceeded. In adult human tissues a variable positive staining was observed in both simple and stratified epithelia. A moderate wolframin expression was observed in liver and in the endocrine portion of the pancreas. In conclusion, our data suggest that this protein may have important roles in a number of different tissues, including many that are not known to be affected by WFS1-linked diseases. The immunopositivity in adult human tissues suggests that it may function maintaining physiological cellular homeostasis.
- Subjects :
- Human adult tissues
Wolfram syndrome
Blotting, Western
Cellular homeostasis
Dot blot
General Biochemistry, Genetics and Molecular Biology
Cell Line
Wolframin
medicine
Humans
Immunohistochemistry
WSF1 gene
Human adult tissue
General Immunology and Microbiology
biology
Membrane Proteins
medicine.disease
human tissue
Cell biology
Blot
Polyclonal antibodies
Cell culture
biology.protein
Antibody
Subjects
Details
- ISSN :
- 19450508 and 19450494
- Database :
- OpenAIRE
- Journal :
- Frontiers in Bioscience
- Accession number :
- edsair.doi.dedup.....824c58e623fad81c9c205a7a2c0d5291