Product Overview
Description
CLPP-00150522 is recombinant human KRT1 protein
Applications
ELISA, SDS-PAGE, Western Blot
Protein Length
Protein fragment
Nature
Recombinant Protein
Sequence
HGDSVRNSKIEISELNRVIQRLRSEIDNVKKQISNLQQSISDAEQRGENALKDAKNKLNDLEDALQQAKEDLARLLRDYQELMNTKLALDLEIATYRTLLEGEESRMSGE
Sequence Similarities
Belongs to the intermediate filament family.
Predicted Molecular Weight
38 kDa including tags
Target Information
Alternative Names
67 kDa cytokeratin; CK-1; CK1; Cytokeratin-1; Cytokeratin1; EHK; EHK1; Epidermolytic hyperkeratosis 1; EPPK; Hair alpha protein; K1; K2C1_HUMAN; Keratin; Keratin type II cytoskeletal 1; Keratin-1; Keratin1; KRT 1; Krt1; KRT1A; NEPPK; type II cytoskeletal 1; Type II keratin Kb1; Type-II keratin Kb1
Protein Function
May regulate the activity of kinases such as PKC and SRC via binding to integrin beta-1 (ITB1) and the receptor of activated protein kinase C (RACK1/GNB2L1).
Tissue Specificity
The source of this protein is neonatal foreskin. The 67-kDa type II keratins are expressed in terminally differentiating epidermis.
Involvement in Disease
Defects in KRT1 are a cause of bullous congenital ichthyosiform erythroderma (BCIE); also known as epidermolytic hyperkeratosis (EHK) or bullous erythroderma ichthyosiformis congenita of Brocq. BCIE is an autosomal dominant skin disorder characterized by widespread blistering and an ichthyotic erythroderma at birth that persist into adulthood. Histologically there is a diffuse epidermolytic degeneration in the lower spinous layer of the epidermis. Within a few weeks from birth, erythroderma and blister formation diminish and hyperkeratoses develop.Defects in KRT1 are the cause of ichthyosis hystrix Curth-Macklin type (IHCM). 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 palmoplantar keratoderma non-epidermolytic (NEPPK). NEPKK is a dermatological disorder characterized by focal palmoplantar keratoderma with oral, genital, and follicular lesions.Defects in KRT1 are a cause of ichthyosis annular epidermolytic (AEI); 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.Defects in KRT1 are the cause of palmoplantar keratoderma striate type 3 (SPPK3); also known as keratosis palmoplantaris striata III. SPPK3 is a dermatological disorder affecting palm and sole skin where stratum corneum and epidermal layers are thickened. There is no involvement of non-palmoplantar skin, and both hair and nails are normal.
Shipping & Handling
Constituents
0.3% Glutathione, 0.79% Tris HCl.
Shipping
Shipped on dry ice.
