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抗体

DKC1多克隆抗体

PA5-75217

100微升

美国446.00

多克隆

domestic rabbit

未共轭

人类, 小鼠, 大鼠

免疫组化, 免疫细胞化学, 免疫组化-石蜡切片

DKC1多克隆抗体

PA5-75217

100微升

美国446.00

多克隆

抗原亲和色谱

兔

人类, 小鼠, 大鼠

Immunocytochemistry: 1:50-1:200, Immunohistochemistry (Paraffin): 1:50-1:200

人类, 小鼠, 大鼠

IgG

短期存放在4°C。 为长期储存, 存放在-20°C, 避免反复冷冻/解冻

This gene is a member of the H/ACA snoRNPs 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 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.

合成多肽对应于氨基酸171-220人的DKC1

液体

Immunocytochemistry: 1:50-1:200, Immunohistochemistry (Paraffin): 1:50-1:200

BC068171; CBF5; CBF5 homolog; DKC; DKC1; DKCX; dyskeratosis congenita 1, dyskerin; dyskeratosis congenita 1, dyskerin homolog; dyskerin; dyskerin pseudouridine synthase 1; H/ACA ribonucleoprotein complex subunit 4; H/ACA ribonucleoprotein complex subunit DKC1; Nap57; NOLA4; Nopp140-associated protein of 57 kDa; nucleolar protein family A member 4; Nucleolar protein NAP57; snoRNP protein DKC1; XAP101