Anti-Desmoglein 2/DSG2 Antibody Picoband® (monoklonale, 2B4D1) fluoro488 Vervoeging

Desmoglein-2 is a protein that in humans is encoded by the DSG2 gene. These desmoglein gene family members are located in a cluster on chromosome 18. This second family member is expressed in colon, colon carcinoma, and other simple and stratified epithelial-derived cell lines. Mutations in DSG2 display a high degree of penetrance. Disease expression was of variable severity with LV involvement a prominent feature. The low prevalence of classical ECG changes highlights the need to expand current diagnostic criteria to take account of LV disease, childhood disease expression, and incomplete penetrance.

Desmin; DES

DSG2

1829

Q14126

Human

No cross-reactivity with other proteins.

E.coli-derived human Desmoglein 2/DSG2 recombinant protein (Position: L24-E1020) .

Clone: 2B4D1

Flow Cytometry

Cancer, Cell Biology, Cell Cycle, Cell Division,2339, Chromosome Structure, DNA/RNA, DNA Damage & Repair, DNA Damage Response, Epigenetics and Nuclear Signaling

Immunogen affinity purified.

Liquid

Muscle-specific type III intermediate filament essential for proper muscular structure and function. Plays a crucial role in maintaining the structure of sarcomeres, inter-connecting the Z-disks and forming the myofibrils, linking them not only to the sarcolemmal cytoskeleton, but also to the nucleus and mitochondria, thus providing strength for the muscle fiber during activity. In adult striated muscle they form a fibrous network connecting myofibrils to each other and to the plasma membrane from the periphery of the Z-line structures. May act as a sarcomeric microtubule-anchoring protein: specifically associates with detyrosinated tubulin-alpha chains, leading to buckled microtubules and mechanical resistance to contraction. Contributes to the transcriptional regulation of the NKX2-5 gene in cardiac progenitor cells during a short period of cardiomyogenesis and in cardiac side population stem cells in the adult. Plays a role in maintaining an optimal conformation of nebulette (NEB) on heart muscle sarcomeres to bind and recruit cardiac alpha-actin.

1. Arnemann J, Spurr NK, Magee AI, Buxton RS (Jul 1992) . The human gene (DSG2) coding for HDGC, a second member of the desmoglein subfamily of the desmosomal cadherins, is, like DSG1 coding for desmoglein DGI, assigned to chromosome 18. Genomics 13 (2) : 484–6. 2. Entrez Gene: DSG2 desmoglein 2. mutations in DSG2 contribute to the development of ARVD/C. 3. Syrris, P., Ward, D., Asimaki, A., Evans, A., Sen-Chowdhry, S., Hughes, S. E., McKenna, W. J. Desmoglein-2 mutations in arrhythmogenic right ventricular cardiomyopathy: a genotype-phenotype characterization of familial disease. Europ. Heart J. 28: 581-588, 2007.

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

6

Desmin

Nucleus. Sarcolemma. Z line. Cytoplasm.

IgG1

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