Recombinant Human Desmoplakin (DSP) Protein (His-SUMO)

Name: Recombinant Human Desmoplakin (DSP) Protein (His-SUMO)
Brand: Beta LifeScience
SKU: BLC-03357P
Availability: InStock

Greater than 90% as determined by SDS-PAGE.

Collections: All products, High-quality recombinant proteins, Other recombinant proteins

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Product Overview

Description	Recombinant Human Desmoplakin (DSP) Protein (His-SUMO) is produced by our E.coli expression system. This is a protein fragment.
Purity	Greater than 90% as determined by SDS-PAGE.
Uniprotkb	P15924
Target Symbol	DSP
Synonyms	250/210 kDa paraneoplastic pemphigus antigen; DCWHKTA; Desmoplakin (DPI DPII); Desmoplakin; Desmoplakin I ; Desmoplakin II; DESP_HUMAN; DP; DP I; DP II; DPI; DPII; DSP; KPPS2; PPKS 2; PPKS2
Species	Homo sapiens (Human)
Expression System	E.coli
Tag	N-6His-SUMO
Target Protein Sequence	CSDCLMRAELIVQPELKYGDGIQLTRSRELDECFAQANDQMEILDSLIREMRQMGQPCDAYQKRLLQLQEQMRALYKAISVPRVRRASSKGGGGYTCQSGSGWDEFTKHVTSECLGWMRQQRAEMDMVAWGVDLASVEQHINSHRGIHNSIGDYRWQLDKIKADLREKSAIYQLEEEYENLLKASFERMDHLRQLQNIIQATSREIMWINDCEEEELLYDWSD
Expression Range	78-300aa
Protein Length	Partial
Mol. Weight	42.1kDa
Research Area	Signal Transduction
Form	Liquid or Lyophilized powder
Buffer	Liquid form: default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.
Reconstitution	Briefly centrifuged the vial prior to opening to bring the contents to the bottom. Reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL. It is recommended to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20°C/-80°C. The default final concentration of glycerol is 50%.
Storage	1. Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. 2. Avoid repeated freeze-thaw cycles. 3. Store working aliquots at 4°C for up to one week. 4. In general, protein in liquid form is stable for up to 6 months at -20°C/-80°C. Protein in lyophilized powder form is stable for up to 12 months at -20°C/-80°C.
Notes	Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.

Target Details

Target Function	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.
Subcellular Location	Cell junction, desmosome. Cytoplasm, cytoskeleton. Cell membrane.
Protein Families	Plakin or cytolinker family
Database References	HGNC: 3052 OMIM: 125647 KEGG: hsa:1832 STRING: 9606.ENSP00000369129 UniGene: PMID: 30207747 For arrhythmogenic right ventricular cardiomyopathy patients, both missense and non-missense desmoplakin mutations carry a high arrhythmic risk. PMID: 28527814 Using molecular dynamics simulations, study elucidated the structural basis of post-translational modifications-induced effects. Simulations, nearing 2 mus in aggregate, indicate that phosphorylation of S2849 induces an "arginine claw" in desmoplakin's C-terminal tail. PMID: 27188911 Study identified DSP to be involved in the progressive intestinal injury associated with the development of Crohn's Disease complications via its effect on intestinal intercellular intermediate filament structure binding. PMID: 28490445 report for the first time in Korea on monozygotic twins with lethal acantholytic epidermolysis bullosa caused by two novel nonsense mutations in the DSP gene PMID: 28442525 Double heterozygotes for mutations in DSP and MYBPC3 showed a variable clinical presentation of arrhythmogenic cardiomyopathy and hypertrophic cardiomyopathy. PMID: 28699631 Study reports a DSP mutation in an association with progressive cardiac conduction disease and a high risk of sudden death. The pathogenicity of the newly identified genetic variant was confirmed by in vitro prediction analysis and familial segregation. These results suggest that the effect of the mutation might be partially due to abnormal function of ion channels as a consequence of desmosomal remodeling. PMID: 27166992 DSP is a desmosomal protein critical to cell-cell adhesion in a variety of cell types and important in wound healing and epithelial barrier function. PMID: 26669357 Novel desmoplakin frameshift deletion p.Thr2625fs (c.7871_7872delAC) was identified as a potential cause of heart disease and sudden cardiac death in a Polish family. PMID: 27698334 This is the first report of DSP genetic screening in Chinese sudden unexplained nocturnal death syndrome (SUNDS) and Brugada syndrome. Our results imply that DSP mutations contribute to the genetic cause of some SUNDS victims and maybe a new susceptible gene for Brugada syndrome. PMID: 26585738 Syndrome featuring erythrokeratodermia and cardiomyopathy (EKC) caused by mutation in DSP was described. Specific region of DSP protein critical to the pathobiology of EKC syndrome and to DSP function in the heart and skin was identified. PMID: 26604139 novel de novo heterozygous missense mutation caused severe dermatitis, multiple allergies, and metabolic wasting syndrome PMID: 26073755 There is higher incidence of Myocarditis in DSP mutation carriers affected by Arrhythmogenic right ventricular dysplasia. PMID: 25616123 Case Reports: desmoplakin mutations associated with variable woolly hair or hypotrichosis, palmoplantar keratoderma, and cardiac manifestations. PMID: 25227139 Genetic testing revealed a novel combination of two heterozygous mutations in the DSP gene encoding desmoplakin PMID: 25516398 Case Report: PKP2/DSP mutations in patient with Brugada syndrome and ventricular tachycardia. PMID: 25900994 Patients whose MPM tissues expressed elevated mRNA levels of BIRC5, DSP, NME2, and THBS2 showed a statistically significant shorter overall survival. PMID: 25771974 GSK3- and PRMT-1-dependent modifications of desmoplakin control desmoplakin-cytoskeleton dynamics. PMID: 25733715 our data indicate that DP phosphorylation at S2849 represents an important mechanism in pemphigus pathogenesis PMID: 25010392 a subset of arrhythmogenic cardiomyopathy mutations, in addition to a mutation associated with skin fragility/woolly hair syndrome, impair gap junction localization and function by misregulating DP-EB1 interactions and altering microtubule dynamics. PMID: 25225338 Arrhythmogenic right ventricular cardiomyopathy patients of a three-generation Spanish family with a novel nonsense genetic variation in the DSP gene. PMID: 23954618 The cardiomyopathy mechanisms varied accordingly to the specific types of DSP mutation identified and included haploinsufficiency, dominant-negative effects, or a combination hereof. PMID: 23137101 DSP is inactivated in lung cancer by an epigenetic mechanism, increases the sensitivity to anticancer drug-induced apoptosis and has tumor-suppressive function, possibly through inhibition of the Wnt/beta-catenin signaling pathway in NSCLC cells. PMID: 22791817 DSP splice variant is crucial for desmosome-mediated adhesion in HaCaT keratinocytes. PMID: 22454510 In two unrelated patients with Naxos-Carvajal syndrome, a single heterozygous de novo mutation in the desmoplakin gene DSP, p.Leu583Pro and p.Thr564Ile, leading to severe phenotypes was found. PMID: 22949226 Desmoplakin disease causes connexin mislocalization in the mouse & man preceding overt histological abnormalities resulting in significant alterations in conduction repolarization kinetics prior to morphological changes detectable on cardiac imaging PMID: 22240500 Desmoplakin and pGSK3beta constitute independent good prognostic factors for progression free survival in Ewing Sarcoma patients. PMID: 22898789 desmoplakin is a promising candidate for supportive CSF marker to rule out 14-3-3 false positive cases in sporadic Creutzfeldt-Jakob disease differential diagnosis. PMID: 22213780 desmoplakin I levels, determined within 1-3 months of the first ischemic stroke, could be a biomarker for statin responsiveness against a new vascular event in post-ischemic stroke patients taking statins PMID: 22304020 Palmitate significantly reduces DSP expression, and treatment with insulin restores the lost expression of DSP. PMID: 22132232 Case Report: desmoplakin mutation responsible for juvenile biventricular cardiomyopathy with left ventricular non-compaction and acantholytic palmoplantar keratoderma. PMID: 21789513 DP Ser2849Gly promotes hyperadhesion PMID: 21993560 We detected a novel mutations: DSP T1373A and it may be associated with a risk for tachycardia PMID: 21397041 A novel nonsense mutation in the desmoplakin gene and the truncated protein which it produces caused left dominant arrhythmogenic cardiomyopathy PMID: 21458130 Mutations in desmoplakin protein is associated with cardiomyopathy. PMID: 21756917 Full sequencing of the DSP showed a novel homozygous c.7097 G>A (p.R2366H) mutation in all skin fragility-woolly hair syndrome affected family members. PMID: 20738328 intimate intramolecular association of the SH3 domain with the preceding SR is also observed in plectin, another plakin protein suggesting that the SH3 domain of plakins contributes to the stability and rigidity of this subfamily of SR-containing proteins PMID: 21536047 SERCA2-deficient cells exhibited up to a 60% reduction in border translocation of desmoplakin (DP), the desmosomal cytolinker protein necessary for intermediate filament (IF) anchorage to sites of robust cell-cell adhesion PMID: 21156808 autosomal-dominant mutations in the DSP gene are associated with hypo/oligodontia in the setting of Carvajal/Naxos syndrome PMID: 20940358 A novel minor isoform of desmoplakin that is also produced by alternative splicing of the desmoplakin gene, is described. PMID: 20524011 we describe a combination of DP mutation phenotypes affecting the skin, heart, hair, and teeth PMID: 19924139 Lethal acantholytic epidermolysis bullosa (LAEB) is an autosomal recessive disorder caused by mutations in the gene encoding the desmosomal protein, desmoplakin (DSP) PMID: 19945626 Compound heterozygosity for non-sense and mis-sense mutations in desmoplakin underlies skin fragility/woolly hair syndrome. haploinsufficiency PMID: 11841538 non-small cell lung carcinoma subtypes have specific patterns of desmoplakin 1 and 2 and cytokeratin 18 gene expression, protein content and biodistribution PMID: 11955647 Mutation (S299R) in exon 7 of desmoplakin causes arrhythmogenic right ventricular cardiomyopathy, unlinked to other causes. PMID: 12373648 desmoplakin has a role in capillary formation PMID: 15190119 Naxos disease is not caused by the desmoplakin gene mutation in an arab family. PMID: 15494820 the involvement of desmoplakin (DP), plectin, and periplakin in the destruction of epithelial cell integrity ensures the efficient elimination of cytoskeleton, but also provides specificity for selectively targeting individual adhesion molecules PMID: 15500642 dominant mutation in desmoplakin that causes left-sided arrhythmogenic left ventricular cardiomyopathywith arrhythmias of left ventricular origin. PMID: 16061754 dramatic phenotype, which we named "lethal acantholytic epidermolysis bullosa," underscores the paramount role of desmoplakin in epidermal integrity PMID: 16175511

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Proteins are sensitive to heat, and freeze-drying can preserve the activity of the majority of proteins. It improves protein stability, extends storage time, and reduces shipping costs. However, freeze-drying can also lead to the loss of the active portion of the protein and cause aggregation and denaturation issues. Nonetheless, these adverse effects can be minimized by incorporating protective agents such as stabilizers, additives, and excipients, and by carefully controlling various lyophilization conditions.

Commonly used protectant include saccharides, polyols, polymers, surfactants, some proteins and amino acids etc. We usually add 8% (mass ratio by volume) of trehalose and mannitol as lyoprotectant. Trehalose can significantly prevent the alter of the protein secondary structure, the extension and aggregation of proteins during freeze-drying process; mannitol is also a universal applied protectant and fillers, which can reduce the aggregation of certain proteins after lyophilization.

Our protein products do not contain carrier protein or other additives (such as bovine serum albumin (BSA), human serum albumin (HSA) and sucrose, etc., and when lyophilized with the solution with the lowest salt content, they often cannot form A white grid structure, but a small amount of protein is deposited in the tube during the freeze-drying process, forming a thin or invisible transparent protein layer.

Reminder: Before opening the tube cap, we recommend that you quickly centrifuge for 20-30 seconds in a small centrifuge, so that the protein attached to the tube cap or the tube wall can be aggregated at the bottom of the tube. Our quality control procedures ensure that each tube contains the correct amount of protein, and although sometimes you can't see the protein powder, the amount of protein in the tube is still very precise.

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