BS1887
antibody from Bioworld Technology, Inc
Targeting: DKC1
Cbf5, DKC, dyskerin, NAP57, NOLA4, XAP101
Antibody data
- Antibody Data
- Antigen structure
- References [0]
- Comments [0]
- Validations
- Western blot [1]
Submit
Validation data
Reference
Comment
Report error
- Product number
- BS1887 - Provider product page
- Provider
- Bioworld Technology, Inc
- Proper citation
- Bioworld Technology Cat#BS1887, RRID:AB_1663571
- Product name
- Dyskerin (K203) polyclonal antibody
- Antibody type
- Polyclonal
- Antigen
- Synthetic peptide, corresponding to amino acids 171-220 of Human Dyskerin.
- Description
- 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
- Reactivity
- Human, Mouse, Rat
- Host
- Rabbit
- Isotype
- IgG
- Vial size
- 100ul
- Concentration
- 1 mg/ml
- Storage
- Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
No comments: Submit comment
Supportive validation
- Submitted by
- Bioworld Technology, Inc (provider)
- Main image
- Experimental details
- Western blot (WB) analysis of Dyskerin (K203) pAb at 1:500 dilutionLane1:A549 cell lysateLane2:Mouse brain tissue lysateLane3:Rat brain tissue lysate