| Catalog number: | bs-13007R-A555 |
|---|---|
| Price: | 332 € |
| Supplier: | Bioss Primary Conjugated Antibodies. |
| Product name: | Dyskerin Antibody, ALEXA FLUOR 555 |
| Quantity: | 100ul |
| Related search: |
| Long name: | ALEXA FLUOR 555 Conjugated, Dyskerin Polyclonal Antibody |
|---|---|
| Also known as: | Anti-Dyskerin PAb ALEXA FLUOR 555 |
| Category: | Conjugated Primary Antibodies |
| Conjugated with: | 555, ALEXA FLUOR® |
| Host Organism: | Rabbit (Oryctolagus cuniculus) |
| Target Antigen: | Dyskerin |
| Specificity: | This is a highly specific antibody against Dyskerin |
| Modification: | Unmodified |
| Modification Site: | None |
| Clonality: | Polyclonal |
| Clone: | Polyclonal antibody |
| Concentration: | 1ug per 1ul |
| Source: | This antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human Dyskerin |
| Gene ID Number: | 1736 |
| Tested applications: | IF(IHC-P) |
| Recommended dilutions: | IF(IHC-P)(1:50-200) |
| Crossreactivity: | Mouse, Rat, Human |
| Cross-reactive species details: | not every possible cross-reactivity is known, Due to limited amount of testing and knowledge |
| Background of the antigen: | DKC is a rare, GAR1, Missense mutations in the dyskerin gene interfere with normal nuclear localization of dyskerin and cause Dyskeratosis congenita (DKC) (4), NHP2 (NOLA2) and Nop10 for the isomerization of uridine to pseudouridine (1), NHP2 and dyskerin localize to the dense fibrillar component of the nucleolus and in nuclear Cajal bodies (2), The GAR1 gene maps to chromosome 4q25 (5, The NHP2 gene maps to chromosome 5q35, The dyskerin gene maps to chromosome Xq28 (3), X-linked bone marrow disorder characterized by cutaneous hyperpigmentation, atrophy of the testicles and leukoplakia of the oral mucosa, dystrophy of the nails, 3 and encodes a 155-amino acid protein (2, 6), 7), Dyskerin (NAP57) associates with the chaperone protein Nopp140 and forms a small ribonucleoprotein particle with GAR1 (NOLA1) |
| Purification: | Purified by Protein A |
| Storage conditions: | 50% glycerol and 0, Keep refrigerated at 2 to 8 degrees Celcius for up to one year, 09% sodium azide, Store this antibody in aqueous buffered solution containing 1% BSA |
| Excitation emission: | 553nm/568nm |
| Synonyms: | XAP101 dyskerin NAP57 NOLA4 Cbf5 |
| Properties: | ALEXA FLUOR they should be stored frozen at - 24°, If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies, Very high photo stable ALEXA conjugate, C, C for long term storage and for short term at + 5°, For facs or microscopy Alexa 1 conjugate |
| Conjugation: | ALEXA FLUOR 555, Alexa Fluor |
| Gene target: | Dyskerin |
| Short name: | Dyskerin Antibody |
| Technique: | antibodies against human proteins, antibodies for, Antibody |
| Label: | ALEXA FLUOR 555 |
| Alternative name: | ALEXA FLUOR 555, Dyskerin (Antibody to) |
| Alternative technique: | antibodies |
| Identity: | 2890 |
| Gene: | DKC1 | More about : DKC1 |
| Long gene name: | dyskerin pseudouridine synthase 1 |
| Synonyms gene: | DKC |
| Synonyms gene name: | dyskeratosis congenita 1, dyskerin |
| Synonyms name: | H/ACA ribonucleoprotein complex subunit 4 |
| Locus: | Xq28 |
| Discovery year: | 2001-06-22 |
| GenBank acession: | AJ224481 |
| Entrez gene record: | 1736 |
| Pubmed identfication: | 9590285 9888995 |
| RefSeq identity: | NM_001363 |
| Classification: | H/ACA ribonucleoprotein complex |
| Havana BLAST/BLAT: | OTTHUMG00000024242 |
| Locus Specific Databases: | DKC1base: Mutation registry for Hoyeraal-Hreidarsson syndrome Mental Retardation database LRG_55 |
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