Dyskerin Antibody

• Catalog number
  bs-13007R
• Price
  Please ask
• Size
  0.1ml

• Long name
  Dyskerin Primary Polyclonal Antibody
• Also known as
  Dyskerin PAb
• Category
  Primary Antibodies
• Conjugation
  Unconjugated
• Target Antigen
  Dyskerin
• Specificity
  This is a highly specific antibody against Dyskerin.
• Modification s
  None
• Modification site s
  Unmodified antibody
• Clonality
  Polyclonal antibody
• Clone number
  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
  WB, IHC-P, IF(IHC-P)
• Recommended dilutions
  WB(1:100-1000), IHC-P(1:100-500), IF(IHC-P)(1:50-200)
• Cross reactivity
  Human, Mouse, Rat
• Cross reactive species details
  Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.
• Background of the target antigen
  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).
• Purification method
  This antibody was purified via Protein A.
• Storage conditions
  Keep the antibody in aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Store at -20°C for up to 1 year.
• Synonym names
  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.
• Properties
  If you buy Antibodies supplied by Bioss Primary Unconjugated Antibodies they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.
• French translation
  anticorps

• Gene target
  Dyskerin  
• Gene symbol
  DKC1
• Short name
  Anti-Dyskerin
• Technique
  Antibody, antibodies against human proteins, antibodies for
• Host
  Rabbit (Oryctolagus cuniculus)
• Isotype
  Immunoglobulin G (IgG)
• Alternative name
  Dyskerin Polyclonal Antibody
• Alternative technique
  antibodies

Gene info

• Identity
  HGNC:2890
• Gene
  DKC1
• Long gene name
  dyskerin pseudouridine synthase 1
• Synonyms gene
  • DKC
• Synonyms gene name
  • dyskeratosis congenita 1, dyskerin
• Synonyms
  • XAP101
  • dyskerin
  • NAP57
  • NOLA4
  • Cbf5
• Synonyms name
  • H/ACA ribonucleoprotein complex subunit 4
• GenBank acession
  • AJ224481
• Locus
  Xq28
• Discovery year
  2001-06-22
• Entrez gene record
  1736
• Pubmed identfication
  • 9590285
  • 9888995
• RefSeq identity
  • NM_001363
• Classification
  • Small nucleolar RNA protein coding host genes
  • Pseudouridine synthases
  • MicroRNA protein coding host genes
  • H/ACA ribonucleoprotein complex
• VEGA ID
  OTTHUMG00000024242
• Locus Specific Databases
  • DKC1base: Mutation registry for Hoyeraal-Hreidarsson syndrome
  • Global Variome shared LOVD
  • LRG_55

MeSH Data

• Name
  Blotting, Western
• Concept
  Scope note: Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes.
• Tree numbers
  • E05.196.401.143
  • E05.301.300.096
  • E05.478.566.320.200
  • E05.601.262
  • E05.601.470.320.200
• Qualifiers
  ethics, trends, veterinary, history, classification, economics, instrumentation, methods, standards, statistics & numerical data

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