DKC1 Polyclonal Antibody

• 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) .

• 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.

• Gene ID:

  1736

• Cellular Locus:

  Cytoplasm, Nucleus

• Host:

  Rabbit

• Immunogen:

  81-190/514

• Target:

  DKC1

• Clonality:

  Polyclonal

• Isotype:

  IgG

• Conjugation:

  Unconjugated

• Source:

  KLH conjugated synthetic peptide derived from human Dyskerin

• Applications:

  WB, ELISA, IHC-P, IHC-F, IF (IHC-P), IF (IHC-F), IF (ICC)

• Purification:

  Purified by Protein A.

• Concentration:

  1µg/µl

• Dilution:

  WB (1:300-5000), ELISA (1:500-1000), IHC-P (1:200-400), IHC-F (1:100-500), IF (IHC-P) (1:50-200), IF (IHC-F) (1:50-200), IF (ICC) (1:50-200)

• Buffer:

  0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

• Modification:

  Unmodified

• Storage Conditions:

  Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

• Gene ID URL:

  1736

• Predicted Cross Reactivity:

  Human, Mouse, Rat, Dog, Cow, Sheep, Pig, Horse, Chicken, Rabbit