catalog number :
MBS6001788
products full name :
Corneodesmosin (CDSN, S Protein)
products short name :
[Corneodesmosin]
products name syn :
[Anti -Corneodesmosin (CDSN, S Protein)]
other names :
[corneodesmosin; Corneodesmosin; corneodesmosin; differentiated keratinocyte S protein; corneodesmosin; S protein]
other gene names :
[CDSN; CDSN; S; PSS; HTSS; HTSS1; D6S586E]
uniprot entry name :
CDSN_HUMAN
sequence :
YLVPGMTYSKGKIYPVGYFTKENPVKGSPGVPSFAAGPP
ISEGKYFSSNP
specificity :
Recognizes human CDSN.
purity :
Affinity Purified. Purified by Protein A affinity chromatography.
form :
Supplied as a liquid in PBS, pH 7.4.
storage stability :
May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
tested application :
ELISA (EL/EIA), Western Blot (WB)
app notes :
Suitable for use in ELISA and Western Blot.
image1 heading :
Western Blot (WB)
image2 heading :
Testing Data
other info1 :
Immunogen: Partial recombinant corresponding to aa306-355 from human CDSN (NP_001255) with GST tag. MW of the GST tag alone is 26kD.
products categories :
Antibodies; Abs to Carbohydrates, Glycoproteins
products description :
CDSN (Corneodesmosin; also S protein) is a 52-56kD, secreted glycoprotein that has an unusually high content of Gly, Pro and Ser. It is found in the desmosomes of spinous layer keratinocytes, and presumably uses its high Gly content to generate homophilic noncovalent intercellular bridges. Mature human CDSN is 497aa in length. It contains a Ser-rich region (aa62-464) and one Gly-rich domain (aa60-171). CDSN undergoes sequential proteolytic processing to generate multiple fragments that vary in size from 46-43kD, to 35-30kD, to 15kD. This sequential extracellular processing allows initially for CDSN incorporation into a functional adhesional junction, and later for the removal of adhesional glycine loops with a subsequent dissociation (desquamation) of cells. There is one potential isoform termed the S protein that shows a two aa substitution for the C-terminal 29aa. Over aa33-529, human CDSN shares 68% aa identity with mouse CDSN.
ncbi acc num :
NP_001255.3
ncbi gb acc num :
NM_001264.4
ncbi mol weight :
51,522 Da
ncbi summary :
This gene encodes a protein found in corneodesmosomes, which localize to human epidermis and other cornified squamous epithelia. During maturation of the cornified layers, the protein undergoes a series of cleavages, which are thought to be required for desquamation. The gene is located in the major histocompatibility complex (MHC) class I region on chromosome 6. [provided by RefSeq, Jul 2008]
uniprot summary :
CDSN: Important for the epidermal barrier integrity. Defects in CDSN are the cause of hypotrichosis type 2 (HYPT2). A condition characterized by the presence of less than the normal amount of hair. Affected individuals have normal hair in early childhood but experience progressive hair loss limited to the scalp beginning in the middle of the first decade and almost complete baldness by the third decade. Body hair, beard, eyebrows, axillary hair, teeth, and nails develop normally. Defects in CDSN are a cause of peeling skin syndrome (PSS); also known as peeling skin syndrome or deciduous skin or keratolysis exfoliativa congenita. A genodermatosis characterized by generalized, continuous shedding of the outer layers of the epidermis. Two main PSS subtypes have been suggested. Patients with non-inflammatory PSS (type A) manifest white scaling, with painless and easy removal of the skin, irritation when in contact with water, dust and sand, and no history of erythema, pruritis or atopy. Inflammatory PSS (type B) is associated with generalized erythema, pruritus and atopy. It is an ichthyosiform erythroderma characterized by lifelong patchy peeling of the entire skin with onset at birth or shortly after. Several patients have been reported with high IgE levels. CDNS mutations are responsible for generalized, inflammatory peeling skin syndrome type B (PubMed:20691404). Protein type: Cell adhesion; Secreted, signal peptide; Secreted. Chromosomal Location of Human Ortholog: 6p21.3. Cellular Component: desmosome; cornified envelope; intercellular junction. Molecular Function: protein homodimerization activity. Biological Process: keratinocyte differentiation; cell-cell adhesion; epidermis development; skin morphogenesis; cell adhesion. Disease: Peeling Skin Syndrome 1; Hypotrichosis 2