DKC1 Polyclonal Antibody

CAT: 0498-bs-13007RSize: 100 µLDry Ice: NoHazardous: No
CAT#:0498-bs-13007RSize:100 µL
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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

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