Recombinant Human Desmoplakin (DSP), partial | CSB-YP007208HU

Recombinant Human Desmoplakin (DSP), partial | CSB-YP007208HU | Cusabio

Alternative Name(s): 250/210KDA paraneoplastic pemphigus antigen

Gene Names: DSP

Research Areas: Signal Transduction

Organism: Homo sapiens (Human)

AA Sequence: CSDCLMRAELIVQPELKYGDGIQLTRSRELDECFAQANDQMEILDSLIREMRQMGQPCDAYQKRLLQLQEQMRALYKAISVPRVRRASSKGGGGYTCQSGSGWDEFTKHVTSECLGWMRQQRAEMDMVAWGVDLASVEQHINSHRGIHNSIGDYRWQLDKIKADLREKSAIYQLEEEYENLLKASFERMDHLRQLQNIIQATSREIMWINDCEEEELLYDWSD

Source: Yeast

Tag Info: N-terminal 6xHis-tagged

Expression Region: 78-300aa

Sequence Info: Partial

MW: 28.1 kDa

Purity: Greater than 90% as determined by SDS-PAGE.

Relevance: 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.

Reference: "Striate palmoplantar keratoderma resulting from desmoplakin haploinsufficiency."Whittock N.V., Ashton G.H., Dopping-Hepenstal P.J., Gratian M.J., Keane F.M., Eady R.A.J., McGrath J.A.J. Invest. Dermatol. 113:940-946(1999)

Storage: The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself. Generally, the shelf life of liquid form is 6 months at -20?/-80?. The shelf life of lyophilized form is 12 months at -20?/-80?.

Notes: Repeated freezing and thawing is not recommended. Store working aliquots at 4? for up to one week.

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.

Involvement in disease: Keratoderma, palmoplantar, striate 2 (SPPK2); Cardiomyopathy, dilated, with woolly hair and keratoderma (DCWHK); Arrhythmogenic right ventricular dysplasia, familial, 8 (ARVD8); Skin fragility-woolly hair syndrome (SFWHS); Epidermolysis bullosa, lethal acantholytic (EBLA); Cardiomyopathy, dilated, with woolly hair, keratoderma, and tooth agenesis (DCWHKTA)

Subcellular Location: Cell junction, desmosome, Cytoplasm, cytoskeleton, Cell membrane

Protein Families: Plakin or cytolinker family

Tissue Specificity: Isoform DPI is apparently an obligate constituent of all desmosomes. Isoform DPII resides predominantly in tissues and cells of stratified origin.

Paythway:

Form: Liquid or Lyophilized powder

Buffer: If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.

Reconstitution: We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20?/-80?. Our default final concentration of glycerol is 50%. Customers could use it as reference.

Uniprot ID: P15924

HGNC Database Link: HGNC

UniGene Database Link: UniGene

KEGG Database Link: KEGG

STRING Database Link: STRING

OMIM Database Link: OMIM