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Rabbit Anti-DSP Recombinant Antibody (CBYCD-428) (V2LY-0425-LY1006)


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Summary

Host Animal
Rabbit
Specificity
Human
Clone
CBYCD-428
Antibody Isotype
IgG
Application
IF

Basic Information

Immunogen
Protein Human DSP (aa 1970-2270).
Host Species
Rabbit
Specificity
Human
Antibody Isotype
IgG
Clonality
Monoclonal Antibody
Application Notes
ApplicationNote
IF(ICC)2 µg/ml

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS
Preservative
Sodium Azide
Concentration
0.5 mg/ml
Purity
>95% as determined by analysis by SDS-PAGE
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Desmoplakin
Entrez Gene ID
1832
UniProt ID
P15924
Research Area
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.
Biological Process
Adherens junction organization Source: Ensembl
Bundle of His cell-Purkinje myocyte adhesion involved in cell communication Source: BHF-UCL
Cell-cell adhesion Source: GO_Central
Desmosome organization Source: BHF-UCL
Epidermis development Source: ProtInc
Epithelial cell-cell adhesion Source: MGI
Intermediate filament cytoskeleton organization Source: GO_Central
Intermediate filament organization Source: BHF-UCL
Keratinocyte differentiation Source: UniProtKB
Peptide cross-linking Source: UniProtKB
Protein localization to cell-cell junction Source: BHF-UCL
Regulation of heart rate by cardiac conduction Source: BHF-UCL
Regulation of ventricular cardiac muscle cell action potential Source: BHF-UCL
Skin development Source: GO_Central
Ventricular compact myocardium morphogenesis Source: BHF-UCL
Wound healing Source: GO_Central
Cellular Location
Cytoskeleton; Cell membrane; Desmosome. Innermost portion of the desmosomal plaque. Colocalizes with epidermal KRT5-KRT14 and simple KRT8-KRT18 keratins and VIM intermediate filaments network (PubMed:12802069). Localizes at the intercalated disk in cardiomyocytes (By similarity).
Involvement in disease
Keratoderma, palmoplantar, striate 2 (SPPK2):
A dermatological disorder characterized by thickening of the skin on the palms (linear pattern) and the soles (island-like pattern) and flexor aspect of the fingers. Abnormalities of the nails, the teeth and the hair are rarely present.
Cardiomyopathy, dilated, with woolly hair and keratoderma (DCWHK):
An autosomal recessive cardiocutaneous syndrome characterized by a generalized striate keratoderma particularly affecting the palmoplantar epidermis, woolly hair, and dilated left ventricular cardiomyopathy.
Arrhythmogenic right ventricular dysplasia, familial, 8 (ARVD8):
A congenital heart disease characterized by infiltration of adipose and fibrous tissue into the right ventricle and loss of myocardial cells, resulting in ventricular and supraventricular arrhythmias.
Skin fragility-woolly hair syndrome (SFWHS):
An autosomal recessive genodermatosis characterized by skin fragility with blistering, focal and diffuse palmoplantar keratoderma, hyperkeratotic plaques on the trunk and limbs, and woolly hair with varying degrees of alopecia.
Epidermolysis bullosa, lethal acantholytic (EBLA):
A form of epidermolysis bullosa characterized by severe fragility of skin and mucous membranes. The phenotype is lethal in the neonatal period because of immense transcutaneous fluid loss. Typical features include universal alopecia, neonatal teeth, and nail loss. Histopathology of the skin shows suprabasal clefting and acantholysis throughout the spinous layer, mimicking pemphigus.
Cardiomyopathy, dilated, with woolly hair, keratoderma, and tooth agenesis (DCWHKTA):
A cardiocutaneous syndrome characterized by biventricular dilated cardiomyopathy, hyperkeratosis, woolly hair, palmoplantar keratoderma, and hypo/oligodontia.
PTM
Ser-2849 is probably phosphorylated by a cAMP-dependent protein kinase. Phosphorylation on Ser-2849 probably affects its association with epidermal, simple cytokeratins and VIM intermediate filaments.
More Infomation

Santos-Ferreira, C., Baptista, R., Teixeira, T., & Gonçalves, L. (2022). A 45-year-old man with sudden cardiac death, cutaneous abnormalities and a rare desmoplakin mutation: a case report and literature review. BMC Cardiovascular Disorders, 22(1), 1-6.

Shoda, T., Kaufman, K. M., Wen, T., Caldwell, J. M., Osswald, G. A., Purnima, P., ... & Rothenberg, M. E. (2021). Desmoplakin and periplakin genetically and functionally contribute to eosinophilic esophagitis. Nature communications, 12(1), 1-15.

Smith, E. D., Lakdawala, N. K., Papoutsidakis, N., Aubert, G., Mazzanti, A., McCanta, A. C., ... & Helms, A. S. (2020). Desmoplakin cardiomyopathy, a fibrotic and inflammatory form of cardiomyopathy distinct from typical dilated or arrhythmogenic right ventricular cardiomyopathy. Circulation, 141(23), 1872-1884.

Hao, Y., Bates, S., Mou, H., Yun, J. H., Pham, B., Liu, J., ... & Zhou, X. (2020). Genome-wide association study: functional variant rs2076295 regulates desmoplakin expression in airway epithelial cells. American journal of respiratory and critical care medicine, 202(9), 1225-1236.

Wang, H., Wu, M., Lu, Y., He, K., Cai, X., Yu, X., ... & Teng, L. (2019). LncRNA MIR4435-2HG targets desmoplakin and promotes growth and metastasis of gastric cancer by activating Wnt/β-catenin signaling. Aging (Albany NY), 11(17), 6657.

Maruthappu, T., Posafalvi, A., Castelletti, S., Delaney, P. J., Syrris, P., O'Toole, E. A., ... & Kelsell, D. P. (2019). Loss‐of‐function desmoplakin I and II mutations underlie dominant arrhythmogenic cardiomyopathy with a hair and skin phenotype. British Journal of Dermatology, 180(5), 1114-1122.

Paller, A. S., Czarnowicki, T., Renert-Yuval, Y., Holland, K., Huynh, T., Sadlier, M., ... & Guttman-Yassky, E. (2018). The spectrum of manifestations in desmoplakin gene (DSP) spectrin repeat 6 domain mutations: Immunophenotyping and response to ustekinumab. Journal of the American Academy of Dermatology, 78(3), 498-505.

Qu, J., Zhu, L., Zhou, Z., Chen, P., Liu, S., Locy, M. L., ... & Zhou, Y. (2018). Reversing mechanoinductive DSP expression by CRISPR/dCas9–mediated epigenome editing. American Journal of Respiratory and Critical Care Medicine, 198(5), 599-609.

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For research use only. Not intended for any clinical use.

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