6
Views
0
CrossRef citations to date
0
Altmetric
Original Article

β-Hexosaminidase in cultured normal and mutant human fibroblasts: an immunohistochemical and biochemical investigation

, , , , &
Pages 711-714 | Received 07 Feb 1991, Accepted 16 Jul 1991, Published online: 08 Jul 2009
 

Abstract

Fibroblasts from a genetically normal individual and mutant fibroblasts from patients with Tay-Sachs and Sandhoffs diseases were grown in vitro. The lysosomal enzyme β-hexosaminidase (Hex) was determined biochemically and localized with monoclonal antibodies recognizing Hex A, and Hex A and Hex B, respectively. The biochemical results showed similar amounts of Hex A and Hex B in the normal fibroblasts, whereas only Hex B activity was detectable in the fibroblasts from the patient with Tay-Sachs disease. The fibroblasts from the patient with Sandhoff's disease showed small amounts of Hex A- and no Hex B activity. Immunohistochemically, Hex was detectable with both antibodies in the normal fibroblasts and in those from the patient with Tay-Sachs disease. The fibroblasts from the patient with Sandhoff's disease were reactive only with Hex A- specific antibody.

Reprints and Corporate Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

To request a reprint or corporate permissions for this article, please click on the relevant link below:

Academic Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

Obtain permissions instantly via Rightslink by clicking on the button below:

If you are unable to obtain permissions via Rightslink, please complete and submit this Permissions form. For more information, please visit our Permissions help page.