Skip to Main Content
 

Global Search Box

 
 
 
 

Files

File List


ysu997808502.pdf (2.42 MB)

ETD Abstract Container

Abstract Header

An Examination of the Levels of Human B-Glucuronidase in a Vohwinkel's Syndrome Patient

Nanosky-Hughes, Monica L.
http://rave.ohiolink.edu/etdc/view?acc_num=ysu997808502

Abstract Details

2001, Master of Science in Biological Sciences, Youngstown State University, Department of Biological Sciences and Chemistry.
Vohwinkel's Syndrome is a rare autosomal dominant epidermolytic palmoplantar keratoderma (EPPK). In 1995, Peris et al described as: 1.) diffuse hyperkeratosis of the palms of the hands and the soles of the feet in which the skin takes on a "honey-comb" appearance. 2.) "star-fish" shaped keratotic plaques on the dorsal surface of the hands and feet, the wrists, knees, and elbows. 3.) fibrous constricting bands (pseudoainhum) at the interphalangeal joints that may eventually lead to autoamputation of that digit. 4.) the invariable appearance of the disease early in life. 5.) and familial incidence (autosomal dominant inheritance pattern). Recently, it was discovered that one of the factors contributing to the disease was a molecular defect in a single protein located in the cornified cell envelope protein, termed loricin (Mastrini, 1996). This defect seemingly impairs the differentiation process of the epidermal renewal system, by not allowing dead skin to slough off. The defect also contributes to barrier function impairment, causing affected patients to retain water in the keratinocytes. It was noted in 1988 by Camisa et al that Vohwinkel's Syndrome patients had an increased level in serum B-glucurondase. B-glucurondase is a lysosomal enzyme known to break down constitutive basement membrane proteins. It has been found in keratinocytes and Langerhan's cells. This study examined the levels of B-glucuronidase in epidermal skin punch biopsies, blood serum samples, and urine specimens from both diseased and non-diseased patients. A quantification of the levels of the enzyme was performed via a double antibody sandwich ELISA. It was hypothesized by this study that perhaps the increased levels of B-glucuronidase was compensatory for the molecular defect in loricrin.
Paul Peterson (Advisor)
75 p.
Biology, General
B-glucuronidase; Vohwinkel's Syndrome

Recommended Citations

Citations

  • Nanosky-Hughes, M. L. (2001). An Examination of the Levels of Human B-Glucuronidase in a Vohwinkel's Syndrome Patient [Master's thesis, Youngstown State University]. OhioLINK Electronic Theses and Dissertations Center. http://rave.ohiolink.edu/etdc/view?acc_num=ysu997808502

    APA Style (7th edition)

  • Nanosky-Hughes, Monica. An Examination of the Levels of Human B-Glucuronidase in a Vohwinkel's Syndrome Patient. 2001. Youngstown State University, Master's thesis. OhioLINK Electronic Theses and Dissertations Center, http://rave.ohiolink.edu/etdc/view?acc_num=ysu997808502.

    MLA Style (8th edition)

  • Nanosky-Hughes, Monica. "An Examination of the Levels of Human B-Glucuronidase in a Vohwinkel's Syndrome Patient." Master's thesis, Youngstown State University, 2001. http://rave.ohiolink.edu/etdc/view?acc_num=ysu997808502

    Chicago Manual of Style (17th edition)

ysu997808502
693
© 2001, all rights reserved.
This open access ETD is published by Youngstown State University and OhioLINK.