Anti-Desmoplakin/DSP Antilichaam Picoband® 31.0647 Vervoeging

Desmoplakin is a protein in humans that is encoded by the DSP gene. This gene encodes a protein that anchors intermediate filaments to desmosomal plaques and forms an obligate component of functional desmosomes. Mutations in this gene are the cause of several cardiomyopathies and keratodermas, including skin fragility-woolly hair syndrome. Alternative splicing results in multiple transcript variants.

Desmoplakin; DP; 250/210 kDa paraneoplastic pemphigus antigen; DSP

DSP

1832

P15924

Human

No cross-reactivity with other proteins.

E.coli-derived human Desmoplakin/DSP recombinant protein (Position: N69-H2195) .

Isoform DPI is apparently an obligate constituent of all desmosomes. Isoform DPII resides predominantly in tissues and cells of stratified origin.

Flow Cytometry

Calcium Binding Proteins, Calcium Signaling, Cell Biology, Cell Cycle, Cell Differentiation, Cytoskeleton/ECM, Developmental Biology, ECM Proteins, Extracellular Matrix, Organogenesis, Signal Transduction, Signaling Pathway, Skeletal Development, Structures

Immunogen affinity purified.

Liquid

Major high molecular weight protein of desmosomes. Involved in the organization of the desmosomal cadherin- plakoglobin complexes into discrete plasma membrane domains and in the anchoring of intermediate filaments to the desmosomes.

1. Choi HJ, Weis WI (2011) . Crystal structure of a rigid four-spectrin-repeat fragment of the human desmoplakin plakin domain. J. Mol. Biol. 409 (5) : 800–12. 2. Green KJ, Parry DA, Steinert PM, Virata ML, Wagner RM, Angst BD, Nilles LA (Feb 1990) . Structure of the human desmoplakins. Implications for function in the desmosomal plaque. The Journal of Biological Chemistry. 265 (5) : 2603–12. 3. Jonkman MF, Pasmooij AM, Pasmans SG, van den Berg MP, Ter Horst HJ, Timmer A, Pas HH (Oct 2005) . Loss of desmoplakin tail causes lethal acantholytic epidermolysis bullosa. American Journal of Human Genetics. 77 (4) : 653–60.

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

57371 MW

6

Desmoplakin

Cell junction, desmosome.

Steroid 17-alpha-hydroxylase/17,20 lyase

Rabbit IgG

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.