Detection of cutaneous prion protein deposits could help diagnose GPI-anchorless prion disease with neuropathy.
Eur J Neurol
; 28(6): 2133-2137, 2021 06.
Article
em En
| MEDLINE
| ID: mdl-33420752
ABSTRACT
BACKGROUND AND PURPOSE:
To investigate prion protein (PrP) deposits in cutaneous tissues of patients of glycosylphosphatidylinositol (GPI)-anchorless prion diseases with neuropathy.METHODS:
Cutaneous tissue samples from three patients with GPI-anchorless prion diseases were obtained, two cutaneous biopsy samples from the lower leg of Case 1 (Y162X) and Case 3 (D178fs25), and a cutaneous sample taken from the abdomen during an autopsy of Case 2 (D178fs25). We performed immunohistochemistry for PrP to look for abnormal PrP deposits.RESULTS:
PrP deposits were observed in the dermal papilla, the sweat glands, the hair follicles, the arrector pili muscles, and peripheral nerves of all examined cases of GPI-anchorless prion disease with neuropathy. The abnormal PrP accumulation was frequently localized at the basement membrane, and colocalized with laminin.CONCLUSION:
Immunohistochemical detection of PrP in cutaneous samples could be used to definitively diagnose GPI-anchorless PrP disease with neuropathy.Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Doenças Priônicas
/
Proteínas Priônicas
Idioma:
En
Ano de publicação:
2021
Tipo de documento:
Article