von Bomhard, Wolf; Mauldin, Elizabeth A; Schmutz, Sheila M; Leeb, Tosso; Casal, Margret L (2006). Black hair follicular dysplasia in Large Münsterländer dogs: clinical, histological and ultrastructural features. Veterinary dermatology, 17(3), pp. 182-8. Oxford: Blackwell Science 10.1111/j.1365-3164.2006.00517.x
Full text not available from this repository.Four Large Münsterländer cross-bred dogs affected with black hair follicular dysplasia (BHFD) and one unaffected control littermate were observed, and skin was sampled weekly over the first 19 weeks of life. Affected dogs were born with silvery grey hair, a consequence of melanin clumping in the hair shafts. Hair bulb melanocytes were densely pigmented, and contained abundant stage IV melanosomes but adjacent matrix keratinocytes lacked melanosomes. Melanin clumping was not prominent in epidermal melanocytes in the haired skin but occurred in the foot pads. Follicular changes progressed from bulbar clumping, clumping in the isthmus/infundibulum and finally to dysplastic hair shafts. Alopecia developed progressively in pigmented areas. Silver-grey hair, melanin clumping, accumulation of stage IV melanosomes within melanocytes and insufficient melanin transfer to adjacent keratinocytes are also classic features of human Griscelli syndrome. The underlying cause in Griscelli syndrome is a defect of melanocytic intracellular transport proteins leading to inadequate and disorganized melanosome transfer to keratinocytes with resultant melanin clumping. In view of the correlation in the phenotype, histology and ultrastructure between both disorders, a defect in intracellular melanosome transport is postulated as the pathogenic mechanism in BHFD.
Item Type: |
Journal Article (Original Article) |
|---|---|
Division/Institute: |
05 Veterinary Medicine > Department of Clinical Research and Veterinary Public Health (DCR-VPH) > Institute of Genetics |
UniBE Contributor: |
Leeb, Tosso |
ISSN: |
0959-4493 |
Publisher: |
Blackwell Science |
Language: |
English |
Submitter: |
Factscience Import |
Date Deposited: |
04 Oct 2013 14:45 |
Last Modified: |
05 Dec 2022 14:13 |
Publisher DOI: |
10.1111/j.1365-3164.2006.00517.x |
PubMed ID: |
16674733 |
Web of Science ID: |
000237353900005 |
URI: |
https://boris.unibe.ch/id/eprint/18463 (FactScience: 606) |
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