Mouse Anti-Human Desmoglein-3 (EC2 Domain) Monoclonal Antibody, Clone QWC39 (CABT-L2646) This Product Is for Research Use Only and Is Not Intended for Diagnostic Use

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Mouse Anti-Human desmoglein-3 (EC2 domain) monoclonal antibody, clone QWC39 (CABT-L2646) This product is for research use only and is not intended for diagnostic use. PRODUCT INFORMATION Product Overview Recombinant monoclonal antibody to desmoglein-3. Variable regions (i.e. specificity) from the EBV transformed human B cell clone QWC39. Made by immortalizing IgG-expressing B cells from Pemphigus vulgaris (PV) patients. Specificity This antibody specifically recognizes a conformational epitope in the EC2 domain of human desmoglein-3 (DSG). This antibody was isolated as a human IgG4 from a patient with mucocutaneous pemphigus vulgaris. This antibody has in vitro and in vivo pathogenic activity, as assessed in a keratinocyte dissociation assay and by the formation of microscopic blisters in newborn mice. This antibody crossreacts with murine DSG-3. Immunogen Desmoglein-3 Isotype IgG1, κ Source/Host Mouse Species Reactivity Human Clone QWC39 Purification Protein A affinity purified Conjugate Unconjugated Applications IA Format Liquid Concentration 1 mg/ml Size 200 ug; 1 mg Buffer PBS Preservative See individual product datasheet Storage Store at -20 or -70°C upon receipt. Divide antibody into aliquots prior usage. Avoid multiple freeze-thaw cycles. Ship Wet ice 45-1 Ramsey Road, Shirley, NY 11967, USA Email: [email protected] Tel: 1-631-624-4882 Fax: 1-631-938-8221 1 © Creative Diagnostics All Rights Reserved Warnings For research use only and not for use in diagnostic or therapeutic procedures. BACKGROUND Introduction Desmosomes are cell-cell junctions between epithelial, myocardial, and certain other cell types. Desmoglein 3 is a calcium-binding transmembrane glycoprotein component of desmosomes in vertebrate epithelial cells. Currently, three desmoglein subfamily members have been identified and all are members of the cadherin cell adhesion molecule superfamily. These desmoglein gene family members are located in a cluster on chromosome 18. This protein has been identified as the autoantigen of the autoimmune skin blistering disease pemphigus vulgaris. Keywords DSG3; desmoglein 3; PVA; CDHF6; desmoglein-3; cadherin family member 6; pemphigus vulgaris antigen; 130-kD pemphigus vulgaris antigen; 130 kDa pemphigus vulgaris antigen; GENE INFORMATION Official Symbol desmoglein 3 Entrez Gene ID 1830 Protein Refseq NP_001935 UniProt ID P32926 Chromosome Location 18q12.1 Pathway Apoptosis; Apoptotic cleavage of cell adhesion proteins; Apoptotic cleavage of cellular proteins; Apoptotic execution phase; Programmed Cell Death; 45-1 Ramsey Road, Shirley, NY 11967, USA Email: [email protected] Tel: 1-631-624-4882 Fax: 1-631-938-8221 2 © Creative Diagnostics All Rights Reserved.

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Supplementary Table 1: Adhesion Genes Data Set
Supplementary Table 1: Adhesion genes data set PROBE Entrez Gene ID Celera Gene ID Gene_Symbol Gene_Name 160832 1 hCG201364.3 A1BG alpha-1-B glycoprotein 223658 1 hCG201364.3 A1BG alpha-1-B glycoprotein 212988 102 hCG40040.3 ADAM10 ADAM metallopeptidase domain 10 133411 4185 hCG28232.2 ADAM11 ADAM metallopeptidase domain 11 110695 8038 hCG40937.4 ADAM12 ADAM metallopeptidase domain 12 (meltrin alpha) 195222 8038 hCG40937.4 ADAM12 ADAM metallopeptidase domain 12 (meltrin alpha) 165344 8751 hCG20021.3 ADAM15 ADAM metallopeptidase domain 15 (metargidin) 189065 6868 null ADAM17 ADAM metallopeptidase domain 17 (tumor necrosis factor, alpha, converting enzyme) 108119 8728 hCG15398.4 ADAM19 ADAM metallopeptidase domain 19 (meltrin beta) 117763 8748 hCG20675.3 ADAM20 ADAM metallopeptidase domain 20 126448 8747 hCG1785634.2 ADAM21 ADAM metallopeptidase domain 21 208981 8747 hCG1785634.2|hCG2042897 ADAM21 ADAM metallopeptidase domain 21 180903 53616 hCG17212.4 ADAM22 ADAM metallopeptidase domain 22 177272 8745 hCG1811623.1 ADAM23 ADAM metallopeptidase domain 23 102384 10863 hCG1818505.1 ADAM28 ADAM metallopeptidase domain 28 119968 11086 hCG1786734.2 ADAM29 ADAM metallopeptidase domain 29 205542 11085 hCG1997196.1 ADAM30 ADAM metallopeptidase domain 30 148417 80332 hCG39255.4 ADAM33 ADAM metallopeptidase domain 33 140492 8756 hCG1789002.2 ADAM7 ADAM metallopeptidase domain 7 122603 101 hCG1816947.1 ADAM8 ADAM metallopeptidase domain 8 183965 8754 hCG1996391 ADAM9 ADAM metallopeptidase domain 9 (meltrin gamma) 129974 27299 hCG15447.3 ADAMDEC1 ADAM-like,
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Desmoglein 3 Anchors Telogen Hair in the Follicle
Journal of Cell Science 111, 2529-2537 (1998) 2529 Printed in Great Britain © The Company of Biologists Limited 1998 JCS3800 Desmoglein 3 anchors telogen hair in the follicle Peter J. Koch, M. G. Mahoney, George Cotsarelis, Kyle Rothenberger, Robert M. Lavker and John R. Stanley* Department of Dermatology, University of Pennsylvania School of Medicine, 211 CRB, 415 Curie Blvd, Philadelphia, PA 19104, USA *Author for correspondence (e-mail: [email protected]) Accepted 6 July; published on WWW 13 August 1998 SUMMARY Little is known about the function of desmosomes in the (acantholysis) between the cells surrounding the telogen normal structure and function of hair. Therefore, it was club and the basal layer of the outer root sheath epithelium. surprising that mice without desmoglein 3 (the autoantigen Electron microscopy revealed ‘half-desmosomes’ at the in pemphigus vulgaris) not only developed mucous plasma membranes of acantholytic cells. Similar membrane and skin lesions like pemphigus patients, but acantholytic histology and ultrastructural ﬁndings have also developed hair loss. Analysis of this phenotype been previously reported in skin and mucous membrane indicated that hair was normal through the ﬁrst growth lesions of DSG3−/− mice and pemphigus vulgaris patients. phase (‘follicular neogenesis’). Around day 20, however, Immunoperoxidase staining with an antibody raised when the hair follicles entered the resting phase of the hair against mouse desmoglein 3 showed intense staining on the growth cycle (telogen), mice with a targeted disruption of cell surface of keratinocytes surrounding the telogen hair the desmoglein 3 gene (DSG3−/−) lost hair in a wave-like club in normal mice.
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Whole Exome Sequencing with Genomic Triangulation Implicates CDH2-Encoded N-Cadherin As a Novel Pathogenic Substrate for Arrhythmogenic Cardiomyopathy
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