DKC1 antibody (AA 81-190) (Biotin)

Catalog No. ABIN1700646

This anti-DKC1 antibody is a Rabbit Polyclonal antibody detecting DKC1 in WB, ELISA, IHC (p) and IHC (fro). Suitable for Human.

Quick Overview for DKC1 antibody (AA 81-190) (Biotin) (ABIN1700646)

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

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This DKC1 antibody is conjugated to Biotin

Application: Western Blotting (WB), ELISA, Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))

Product Details anti-DKC1 Antibody (Biotin)

Binding Specificity: AA 81-190

Predicted Reactivity: Human,Mouse,Rat,Dog,Cow,Sheep,Pig,Horse,Chicken,Rabbit

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human Dyskerin

Isotype: IgG

Application Details

Application Notes: WB 1:300-5000
IHC-P 1:200-400
IHC-F 1:100-500

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Preservative: ProClin

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

Storage: -20 °C

Storage Comment: Store at -20°C for 12 months.

Expiry Date: 12 months

Target Details for DKC1

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

Alternative Name: Dyskerin

Background:

Synonyms: CBF5, CBF5 homolog, Cbf5p homolog, DKC 1, DKC, Dkc1, DKC1_HUMAN, DKCX, Dyskeratosis congenita 1, Dyskeratosis congenita 1 dyskerin, Dyskerin, H/ACA ribonucleoprotein complex subunit 4, NAP 57, NAP57, NAP-57, NOLA 4, NOLA4, Nopp140 associated protein of 57 kDa, Nopp140-associated protein of 57 kDa, Nucleolar protein family A member 4, Nucleolar protein NAP57, snoRNP protein DKC1, XAP 101, XAP101.

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 (1). GAR1, NHP2 and dyskerin localize to the dense fibrillar component of the nucleolus and in nuclear Cajal bodies (2). The dyskerin gene maps to chromosome Xq28 (3). Missense mutations in the dyskerin gene interfere with normal nuclear localization of dyskerin and cause Dyskeratosis congenita (DKC) (4). 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 (5,6). The NHP2 gene maps to chromosome 5q35.3 and encodes a 155-amino acid protein (2,7).

Gene ID: 1736

Pathways: Telomere Maintenance