DKC1 antibody-多抗-深圳欣博盛生物科技有限公司

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DKC1 antibody

品牌：GeneTex

官网链接

说明书

折扣/货期

货号：GTX109000	规格：100μl	目录价：￥4000
货号：GTX109000-S	规格：25μl	目录价：￥1700

产品详情

* 以下信息仅供参考，详情请以原厂网站为准

产品名称:

DKC1 antibody

别名:

dyskerin pseudouridine synthase 1 , CBF5 , DKC , DKCX , NAP57 , NOLA4 , XAP101

反应种属:

Human

宿主来源:

Rabbit

实验应用:

ICC/IF, IHC-P, IP, WB

靶标/特异性:

KO/KD validation is based on published data (PMID: 26990993).

同种型:

IgG

免疫原:

Recombinant protein encompassing a sequence within the center region of human DKC1. The exact sequence is proprietary.

克隆性:

Polyclonal

克隆号:

纯化方式:

Purified by antigen-affinity chromatography.

偶联:

Unconjugated

产品浓度:

1 mg/ml (Please refer to the vial label for the specific concentration.)

保存温度:

Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.

运输温度:

4°C

预期分子量:

产品形式:

Liquid

存储溶液:

PBS, 1% BSA, 20% Glycerol, 0.01% Thimerosal.

生产商:

GeneTex

功能与背景:

This gene 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. Mutations in this gene also cause Hoyeraal-Hreidarsson syndrome, which is a more severe form of dyskeratosis congenita. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq]

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