DKC1 antibody (AA 171-220)

Catalog No. ABIN5540295

The Rabbit Polyclonal anti-DKC1 antibody is suitable to detect DKC1 in samples from Human, Mouse and Rat. It has been validated for WB, IF, ICC and IHC (p).

Quick Overview for DKC1 antibody (AA 171-220) (ABIN5540295)

Target: DKC1 (Dyskeratosis Congenita 1, Dyskerin (DKC1))

Reactivity: Human, Mouse, Rat

Host: Rabbit

Clonality: Polyclonal

Conjugate: This DKC1 antibody is un-conjugated

Application: Western Blotting (WB), Immunofluorescence (IF), Immunocytochemistry (ICC), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Product Details anti-DKC1 Antibody

Binding Specificity: AA 171-220

Specificity: The antibody detects endogenous levels of Dyskerin protein. (region surrounding Lys203)

Purification: Affinity Chromatography using epitope-specific immunogen

Immunogen: Synthetic peptide, corresponding to amino acids 171-220 of Human Dyskerin.

Application Details

Application Notes: Western blot: 1/500-1/1000. Immunofluorescence : 1/50-1/200. Immunohistochemistry on Paraffin Sections: 1/50-1/200.

Restrictions: For Research Use only

Handling

Format: Liquid

Buffer: 95 % by SDS-PAGE) Aff - Purified

Preservative: Sodium azide

Precaution of Use: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Storage: 4 °C,-20 °C

Storage Comment: Store undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing. Shelf life: one year from despatch.

Expiry Date: 12 months

Target Details for DKC1

Target: DKC1 (Dyskeratosis Congenita 1, Dyskerin (DKC1))

Alternative Name: dkc1,nola4

Background: Dyskerin (NAP57) associates with the chaperone protein Nopp140 and forms a small ribonucleoprotein particle with GAR1 (NOLA1), NHP2 (NOLA2) and Nop10 for the isomerization of uridine to pseudouridine. GAR1, NHP2 and Dyskerin localize to the dense fibrillar component of the nucleolus and in nuclear Cajal bodies. The Dyskerin gene maps to chromosome Xq28. Missense mutations in the Dyskerin gene interfere with normal nuclear localization of Dyskerin and cause Dyskeratosis congenita (DKC). DKC is a rare, X-linked bone marrow disorder characterized by cutaneous hyperpigmentation, dystrophy of the nails, atrophy of the testicles and leukoplakia of the oral mucosa. The GAR1 gene maps to chromosome 4q25 and encodes a 28 kDa protein. The NHP2 gene maps to chromosome 5q35.3 and encodes a 155 amino acid protein.

UniProt: O60832

Pathways: Telomere Maintenance