Anti-Dyskerin (Polyclonal), ALEXA Fluor 594

• Catalog number
  GENTObs-13007R-A594
• Price
  Please ask
• Size
  100 microliters

• Type
  Conjugated Primary Antibody
• Conjugated with
  ALEXA FLUOR® 594
• Host organism
  Rabbit (Oryctolagus cuniculus)
• Target Protein Peptide
  Dyskerin
• Specificity
  This antibody reacts specifically with Dyskerin
• Modification
  No modification has been applied to this antibody
• Modification site
  None
• Clonality
  Polyclonal Antibody
• Clone
  Polyclonal Antibodies
• Concentration
  1ug per 1ul
• Subcellular locations
  N/A
• Antigen Source
  KLH conjugated synthetic peptide derived from human Dyskerin
• Gene ID
  1736
• Swiss Prot
  N/A
• Applications
  IF(IHC-P)
• Applications with corresponding dilutions
  IF(IHC-P)(1:50-200)
• Cross reactive species
  Human (Homo sapiens), Mouse (Mus musculus), Rat (Rattus norvegicus)
• Cross Reactive Species details
  No significant cross reactivity has been observed for this antibody for the tested species. However, note that due to limited knowledge it is impossible to predict with 100% guarantee that the antibody does not corss react with any other species.
• Background information
  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
  Purified by Protein A.
• Storage
  Water buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at 4°C for 12 months.
• Excitation emission
  590nm/617nm
• 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.
• Also known as
  Dyskerin Polyclonal Antibody
• Other name
  Anti-Dyskerin Polyclonal
• Advisory
  Avoid freeze/thaw cycles as they may denaturate the polypeptide chains of the antibody, thus reducing its reactivity, specificity and sensitivity. For antibodies that are in liquid form or reconstituted lyophilized antibodies small amounts could become entrapped on the seal or the walls of the tube. Prior to use briefly centrifuge the vial to gather all the solution on the bottom.
• Properties
  For facs or microscopy Alexa 1 conjugate.
• Conjugation
  Alexa Fluor, ALEXA FLUOR® 594
• Conjugated
  Alexa conjugate 1
• Description
  This antibody needs to be stored at + 4°C in a fridge short term in a concentrated dilution. Freeze thaw will destroy a percentage in every cycle and should be avoided. Antibody for research use.
• Group
  Polyclonals and antibodies
• About
  Polyclonals can be used for Western blot, immunohistochemistry on frozen slices or parrafin fixed tissues. The advantage is that there are more epitopes available in a polyclonal antiserum to detect the proteins than in monoclonal sera.

• Gene target
  Dyskerin  
• Gene symbol
  DKC1
• Short name
  Anti-Dyskerin
• Technique
  Polyclonal, anti-, anti, antibody to, antibodies, Polyclonal antibodies (pAbs) are mostly rabbit or goat antibodies that are secreted by different B cells, whereas monoclonal antibodies come from a single N cell lineage. Pabs are a collection of immunoglobulin molecules that react against a specific antigen, each identifying a different epitope.
• Isotype
  Immunoglobulin G (IgG)
• Label
  ALEXA FLUOR 594
• Alternative name
  Dyskerin Polyclonal Antibody, ALEXA FLUOR® 594 Conjugated
• Alternative technique
  polyclonals, 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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