Mouse Desmoplakin 1,2 Monoclonal Antibody | anti-DSP antibody

Desmoplakin 1,2

Cat. Num. AAA608063

• Gene Names: DSP; DP; DPI; DPII
• Reactivity: Bovine, Chicken, Human, Mouse, Rat
• Applications: Western Blot, Immunohistochemistry, Immunofluorescence
• Purity: Affinity Purified; Purified by Protein A affinity chromatography.
• Synonyms: Desmoplakin 1; 2; Monoclonal Antibody; Anti -Desmoplakin 1; anti-DSP antibody

• Host: Mouse
• Reactivity: Bovine, Chicken, Human, Mouse, Rat
• Clonality: Monoclonal
• Isotype: IgG1
• Clone Number: 5F190
• Specificity: Recognizes bovine desmoplakin 1 and 2 at ~210 and 215kD. Reacts with the primary and metastatic carcinoma and meningioma. Species Crossreactivity: human, rat, mouse and chicken.
• Purity/Purification: Affinity Purified; Purified by Protein A affinity chromatography.
• Form/Format: Supplied as a lyophilized powder from PBS, pH 7.4, 0.5% BSA, 0.09% sodium azide. Reconstitute with 1ml ddH2O.

• Applicable Applications for anti-DSP antibody: Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)
• Application Notes: Suitable for use in Western Blot, Immunofluorescence and Immunohistochemistry.; Dilution: Immunohistochemistry (Frozen): 1:10 with PBS, pH 7.4; Incubation Time 1 hr at RT.
• Immunogen: Bovine desmoplakin 1&2
• Preparation and Storage: Lyophilized powder may be stored at -20 degree C. Stable for 12 months at -20 degree C. Reconstitute with ddH2O. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Reconstituted product is stable for 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

Related Product Information for anti-DSP antibody

Desmoplakin (DP) is a cytoplasmic plaque protein that is located in desmosomes, which are abundant in tissues subjected to mechanical stress, like muscle and epidermis. The role of DP is to link the transmembrane cadherins via plakoglobin to the cytoplasmic intermediate filament network (Kowalczyk et al. 1997; Smith and Fuchs 1998). DP comprises an N-terminal, desmosome-associated plakin domain binding to plakoglobin and plakophilin (Smith and Fuchs 1998), a central coiled-coil rod domain responsible for dimerization, and a C-terminal domain consisting of three homologous plakin repeat subdomains, named "A," "B," and "C," that interact with the intermediate filaments (Choi et al. 2002). Alternative splicing generates two isoforms (DP I and DP II) that differ in their rod domain lengths, with relative molecular weights of 332 kD and 259 kD, respectively (Green et al. 1988).

Mutations in desmoplakin can cause cardiocutaneous syndromes (for review, see Cheong et al. 2005). Haplotype insufficiency of DP may cause striate palmoplantar keratoderma (PPK [MIM 125647]) (Armstrong et al. 1999; Whittock et al. 1999), whereas dominant missense mutations in the N-terminal domain may cause arrhythmogenic right ventricular dysplasia (ARVD [MIM 607450]) (Rampazzo et al. 2002). A nonsense mutation in the N-terminal domain combined with a recessive missense mutation causes skin fragility/woolly hair syndrome with striate PPK [MIM 607655] (Whittock et al. 2002), whereas a homozygous recessive missense mutation in the B-subdomain causes ARVD, woolly hair, and acral skin blistering (Naxos-like disease) (Alcalai et al. 2003), and a homozygous truncatious mutation in the C-terminus causes left ventricular cardiomyopathy, woolly hair, and PPK (Carvajal syndrome [MIM 605676]) (Norgett et al. 2000). DP must be indispensable, because DP-/- mice are early abortive (Gallicano et al. 1998).

Product Categories/Family for anti-DSP antibody

Antibodies; Abs to Proteins

NCBI and Uniprot Product Information

• NCBI GI #: 58530840
• NCBI GeneID: 1832
• NCBI Accession #: NP_004406.2
• NCBI GenBank Nucleotide #: NM_004415.2
• UniProt Accession #: P15924; O75993; Q14189; Q9UHN4; B2RTT2; Q4LE79; Q6ZP16
• Molecular Weight: 331,774 Da
• NCBI Official Full Name: desmoplakin isoform I
• NCBI Official Synonym Full Names: desmoplakin
• NCBI Official Symbol: DSP
• NCBI Official Synonym Symbols: DP; DPI; DPII
• NCBI Protein Information: desmoplakin; desmoplakin I; desmoplakin II; OTTHUMP00000016001; OTTHUMP00000221297; 250/210 kDa paraneoplastic pemphigus antigen
• UniProt Protein Name: Desmoplakin
• Protein Family: Desmoplakin
• UniProt Gene Name: DSP
• UniProt Entry Name: DESP_HUMAN

NCBI Description

Desmosomes are intercellular junctions that tightly link adjacent cells. Desmoplakin is an obligate component of functional desmosomes that anchors intermediate filaments to desmosomal plaques. The N-terminus of desmoplakin is required for localization to the desmosome and interacts with the N-terminal region of plakophilin 1 and plakoglobin. The C-terminus of desmoplakin binds with intermediate filaments. In the mid-region of desmoplakin, a coiled-coiled rod domain is responsible for homodimerization. Mutations in this gene are the cause of several cardiomyopathies and keratodermas as well as the autoimmune disease paraneoplastic pemphigus. [provided by RefSeq]

Uniprot Description

Desmoplakin: 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. Homodimer. Interacts with COL17A1 (via cytoplasmic region). Associates (via C-terminal) with KRT5-KRT14 (via rod region), KRT8-KRT18 and VIM intermediate filaments. Interacts with DSC2. Isoform DPI is apparently an obligate constituent of all desmosomes. Isoform DPII resides predominantly in tissues and cells of stratified origin. Belongs to the plakin or cytolinker family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Cytoskeletal; Cell adhesion; Motility/polarity/chemotaxis

Chromosomal Location of Human Ortholog: 6p24

Cellular Component: desmosome; mitochondrion; basolateral plasma membrane; plasma membrane; intermediate filament; cornified envelope; fascia adherens; nucleus

Molecular Function: protein binding, bridging; protein binding; protein kinase C binding; structural constituent of cytoskeleton; cell adhesion molecule binding; structural molecule activity

Biological Process: keratinocyte differentiation; epidermis development; cell-cell adhesion; wound healing; apoptosis; intermediate filament organization; peptide cross-linking; cell structure disassembly during apoptosis

Disease: Cardiomyopathy, Dilated, With Woolly Hair And Keratoderma; Arrhythmogenic Right Ventricular Dysplasia, Familial, 8; Keratosis Palmoplantaris Striata Ii; Cardiomyopathy, Dilated, With Woolly Hair, Keratoderma, And Tooth Agenesis; Skin Fragility-woolly Hair Syndrome; Epidermolysis Bullosa, Lethal Acantholytic