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Dyskerin Polyclonal Antibody

说明书

BYab-01670

  • 20UL ¥460 50UL ¥945 100UL ¥1350
  • 货期: 3天左右

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宿主
反应性
应用
WB;IHC;IF;ELISA
分子量(DA)
57kD
免疫原
The antiserum was produced against synthesized peptide derived from human Dyskerin. AA range:171-220
特异性
Dyskerin Polyclonal Antibody detects endogenous levels of Dyskerin protein.
来源
Polyclonal, Rabbit,IgG
组成(Formulation)
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
稀释比例
Western Blot: 1/500 - 1/2000. Immunohistochemistry: 1/100 - 1/300. Immunofluorescence: 1/200 - 1/1000. ELISA: 1/40000. Not yet tested in other applications.
纯化工艺(Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
浓度
1 mg/ml
背景(Background)
dyskerin pseudouridine synthase 1(DKC1) Homo sapiens This gene functions in two distinct complexes. It plays an active role in telomerase stabilization and maintenance, as well as recognition of snoRNAs containing H/ACA sequences which provides stability during biogenesis and assembly into H/ACA small nucleolar RNA ribonucleoproteins (snoRNPs). This gene is highly conserved and widely expressed, and may play additional roles in nucleo-cytoplasmic shuttling, DNA damage response, and cell adhesion. Mutations have been associated with X-linked dyskeratosis congenita. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2014],
功能
catalytic activity:RNA uridine = RNA pseudouridine.,disease:Defects in DKC1 are a cause of dyskeratosis congenita X-linked recessive (XDKC) [MIM:305000]. XDKC is a rare, progressive bone marrow failure syndrome characterized by the triad of reticulated skin hyperpigmentation, nail dystrophy, and mucosal leukoplakia. Early mortality is often associated with bone marrow failure, infections, fatal pulmonary complications, or malignancy.,disease:Defects in DKC1 are the cause of Hoyeraal-Hreidarsson syndrome (HHS) [MIM:300240]. HHS is a multisystem disorder affecting males and is characterized by aplastic anemia, immunodeficiency, microcephaly, cerebellar hypoplasia, and growth retardation.,function:Required for ribosome biogenesis and telomere maintenance. Probable catalytic subunit of H/ACA small nucleolar ribonucleoprotein (H/ACA snoRNP) complex, which catalyzes pseudouridylation of rRNA.
基因名称(Gene Name)
DKC1
蛋白名称
H/ACA ribonucleoprotein complex subunit 4
简称
Dyskerin
其他名称
DKC1; NOLA4; H/ACA ribonucleoprotein complex subunit 4; CBF5 homolog; Dyskerin; Nopp140-associated protein of 57 kDa; Nucleolar protein NAP57; Nucleolar protein family A member 4; snoRNP protein DKC1
Fields
>>Ribosome biogenesis in eukaryotes
人基因ID
1736
人蛋白质序列数据库
O60832
小鼠基因ID
245474
小鼠蛋白质序列数据库
Q9ESX5
大鼠基因ID
170944
大鼠蛋白质序列数据库
P40615
细胞定位
[Isoform 1]: Nucleus, nucleolus . Nucleus, Cajal body . Also localized to Cajal bodies (coiled bodies). .; [Isoform 3]: Cytoplasm .
组织表达
Ubiquitously expressed.
储存(Storage)
-20°C/1 year

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Dyskerin Polyclonal Antibody

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