DKC1 antibody (AA 185-213)
Quick Overview for DKC1 antibody (AA 185-213) (ABIN651670)
Target
See all DKC1 AntibodiesReactivity
Host
Clonality
Conjugate
Application
Clone
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Binding Specificity
- AA 185-213
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Purification
- This antibody is purified through a protein A column, followed by peptide affinity purification.
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Immunogen
- This DKC1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 185-213 amino acids from the Central region of human DKC1.
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Isotype
- Ig Fraction
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Application Notes
- IF: 1:10~50. WB: 1:1000. IHC-P: 1:50~100
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Restrictions
- For Research Use only
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Format
- Liquid
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Buffer
- Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) sodium azide.
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Preservative
- Sodium azide
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Precaution of Use
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Storage
- 4 °C,-20 °C
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Storage Comment
- Maintain refrigerated at 2-8 °C for up to 6 months. For long term storage store at -20 °C in small aliquots to prevent freeze-thaw cycles.
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Expiry Date
- 6 months
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- DKC1 (Dyskeratosis Congenita 1, Dyskerin (DKC1))
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Alternative Name
- DKC1
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Background
- DKC1 is a member of the H/ACA snoRNPs (small nucleolar ribonucleoproteins) gene family. snoRNPs are involved in various aspects of rRNA processing and modification and have been classified into two families: C/D and H/ACA. The H/ACA snoRNPs also include the NOLA1, 2 and 3 proteins. The protein encoded by this gene and the three NOLA proteins localize to the dense fibrillar components of nucleoli and to coiled (Cajal) bodies in the nucleus. Both 18S rRNA production and rRNA pseudouridylation are impaired if any one of the four proteins is depleted. These four H/ACA snoRNP proteins are also components of the telomerase complex. The protein encoded by this gene is related to the Saccharomyces cerevisiae Cbf5p and Drosophila melanogaster Nop60B proteins. The gene lies in a tail-to-tail orientation with the palmitoylated erythrocyte membrane protein gene and is transcribed in a telomere to centromere direction. Both nucleotide substitutions and single trinucleotide repeat polymorphisms have been found in this gene. Mutations in this gene cause X-linked dyskeratosis congenita, a disease resulting in reticulate skin pigmentation, mucosal leukoplakia, nail dystrophy, and progressive bone marrow failure in most cases.
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Molecular Weight
- 57674
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Gene ID
- 1736
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NCBI Accession
- NP_001354
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UniProt
- O60832
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Pathways
- Telomere Maintenance
Target
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