Cytokeratin [MNF116]

GWB-3835D9

IgG1Light chain: kappa

Human

Skin

Tissue culture supernatant Storage

For IHC-P: Use at a dilution of 1:25-1:50 in an ABC method. Suggested incubation period of 30mins at RT. Enzymatic predigestion of paraffin embedded tissues is recommended prior to the immunostaining. Not tested in other applications. Optimal dilutions/concentrations should be determined by the researcher. Cytokeratins a group of at least 29 different proteins are characteristic of epithelial and trichocytic cells. Cytokeratins 4 5 6 and 8 are members of the type II neutral-to-basic subfamily. Cytokeratin peptide 4 (59 kDa) is the secondary type II keratin expressed in non cornified stratified squamous epithelia. Cytokeratin peptide 5 (58 kDa) is the primary type II keratin in stratified epithelia while cytokeratin type 8 (52 kDa) is a major type II keratin in simple epithelia. Cytokeratin 6 (56 kDa) is a \" hyperproliferation\" cytokeratin expressed in tissues with natural or pathological high turnover. Cytokeratins 10 13 and 18 are members of the type I acidic subfamily. Cytokeratin peptide 10 (56 kDa) is the secondary type I keratin expressed in cornified epithelia. Cytokeratin 13 (54 kDa) is the secondary type I keratin expressed in non-cornified stratified squamous epithelia. Cytokeratin 18 (45 kDa) is the primary type I keratin expressed in simple epithelial cells. Cytokeratin is a heterotetramer of two type I and two type II keratins. Keratin 1 is generally associated with keratin 10. Defects in Cytokeratin are a cause of epidermolytic hyperkeratosis also known as bullous congenital ichthyosiform erythroderma (BIE) which is a hereditary skin disorder characterized by blistering and a marked thickening of the stratum corneum. Defects in Cytokeratin are also the cause of Curth Macklin type ichthyosis hystrix nonepidermolytic palmoplantar keratoderma annular epidermolytic ichthyosis and keratosis palmoplantaris striata III (PPKS3). PPKS3 is an autosomal dominant disorder affecting palm and sole skin where stratum corneum and epidermal layers are thickened. There is no involvement of nonpalmoplantar skin and both hair and nails are normal.

Heterotetramer of two type I and two type II keratins. Keratin-1 is generally associated with keratin-10. Interacts with ITGB1 in the presence of GNB2L1 and SRC and with GNB2L1.

The source of this protein is neonatal foreskin. The 67-kDa type II keratins are expressed in terminally differentiating epidermis.

There are two size variants of KRT1 termed allele 1A and allele 1B with allelic frequencies of 0. 61 and 0. 39. Allele 1B lacks 7 residues compared to allele 1A.

Defects in KRT1 are the cause of ichthyosis hystrix Curth-Macklin type (IHCM) [MIM:146590]. IHCM is a genodermatosis with severe verrucous hyperkeratosis. Affected individuals manifest congenital verrucous black scale on the scalp neck and limbs with truncal erythema palmoplantar keratoderma and keratoses on the lips ears nipples and buttocks.

Defects in KRT1 are a cause of ichthyosis annular epidermolytic (AEI) [MIM:607602]; also known as cyclic ichthyosis with epidermolytic hyperkeratosis. AEI is a skin disorder resembling bullous congenital ichthyosiform erythroderma. Affected individuals present with bullous ichthyosis in early childhood and hyperkeratotic lichenified plaques in the flexural areas and extensor surfaces at later ages. The feature that distinguishes AEI from BCIE is dramatic episodes of flares of annular polycyclic plaques with scale which coalesce to involve most of the body surface and can persist for several weeks or even months.

There are two types of cytoskeletal and microfibrillar keratin: I (acidic; 40-55 kDa) and II (neutral to basic; 56-70 kDa).