WebJun 27, 2024 · Dystonia musculorum (dt) is a hereditary neurodegenerative disease in mice that leads to sensory ataxia (see ANIMAL MODEL). Brown et al. (1995) cloned a candidate gene for mouse dt, called dystonin, that was predominantly expressed in the dorsal root ganglia and other sites of neurodegeneration in dt mice. They showed that the dystonin … WebFeb 17, 2016 · Dystonin is a cytoskeletal linker protein with both actin- and tubulin-binding domains. Loss of function of this protein results in a sensory neuropathy called Hereditary Sensory Autonomic Neuropathy VI in humans and dystonia musculorumin mice. This disease presents with severe ataxia, dystonic muscle and is ultimately fatal early in life.
Dystonin localization in HFFF2 cells. (A) shControl or …
WebDystonin (DST), also known as bullous pemphigoid antigen 1 (BPAG1), isoforms 1/2/3/4/5/8, is a protein that in humans is encoded by the DST gene. [3] [4] [5] This gene encodes a member of the plakin protein family of adhesion junction plaque proteins. WebDissociation reagents include naturally-occurring enzymes, gentler non-enzymatic alternatives, or may work by chelating calcium to prevent cadherins from attaching, … chinnock hollow
StainExpress™ Immune Cell Composition Cocktail, human
WebHome AHA/ASA Journals WebImmunocytochemistry/ Immunofluorescence: Dystonin Antibody (1B10) [H00000667-M01] - Analysis of monoclonal antibody to DST on HeLa cell . Antibody concentration 10 ug/ml. … WebJun 1, 1998 · A central role for the Schwann cell cytoskeleton in the process of peripheral nerve myelination has long been suggested. However, there is no genetic or biological evidence as yet to support this assumption. Here we show that dystonia musculorum (dt) mice, which carry mutations in dystonin, a cytoskeletal crosslinker protein, have … granite like countertops