Keratin

GWB-61F21E

Keratin isolated from fetal bovine hooves.

This product does not react with mesenchymal glial neuronal or muscle cells.

Affinity purified rabbit sera. Storage

IF: Use at a dilution of 1/20. Not tested in other applications. Optimal dilutions/concentrations should be determined by the end user. A wide variety of cells contain in addition to microtubules and microfilaments another type of fibrous skeletal element which are classified under the category of \" \" intermediate-sized\" \" filaments. These intermediate sized filaments are stable in vivo and show a biochemical heterogeneity which is developmentally regulated. Each class of intermediate filament is restricted in vivo by tissue differentiation; keratins to epithelia vimentin to mesenchymal cells desmin to muscle tissues glial fibrillary acidic protein (GFAP) to astroglia and neurofilament proteins to neurones. Unlike actin and tubulin where molecular size is highly conserved from cell to cell and species to species intermediate filament proteins vary greatly in size from class to class as well as species to species.

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).