bs-13007R-Cy7

DATASHEET

Host: Rabbit

Target Protein: Dyskerin

Immunogen Range: 81-190/514

Clonality: Polyclonal

Isotype: IgG

Entrez Gene: 1736

Source: KLH conjugated synthetic peptide derived from human Dyskerin

Purification: Purified by Protein A.

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

Storage: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

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).

Conjugation: Cy7

Excitation/ Emission: 743nm/767nm

Size: 100ul

Concentration: 1ug/ul

Applications: WB(1:300-5000)
IF(IHC-P)(1:50-200)
IF(IHC-F)(1:50-200)
IF(ICC)(1:50-200)

Predicted Molecular Weight: 58

Predicted Cross Reactive Species: Human
Mouse
Rat
Dog
Cow
Sheep
Pig
Horse
Chicken
Rabbit

For research use only. Not intended for diagnostic or therapeutic use.