H/ACA ribonucleoprotein complex subunit 4 (Dkc1) Recombinant Protein | Dkc1 recombinant protein
Recombinant Rat H/ACA ribonucleoprotein complex subunit 4 (Dkc1)
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. This protein 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. This protein 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.
NCBI and Uniprot Product Information
NCBI Description
nucleolar protein expressed in liver; colocalized with Nopp140 protein; may be involved with rRNA processing [RGD, Feb 2006]
Uniprot Description
Required for telomere maintenance; necessary for correct processing or intranuclear trafficking of TERC, the RNA component of the telomerase reverse transcriptase (TERT) holoenzyme (). Also required for ribosome biogenesis. Probable catalytic subunit of H/ACA small nucleolar ribonucleoprotein (H/ACA snoRNP) complex, which catalyzes pseudouridylation of rRNA. This involves the isomerization of uridine such that the ribose is subsequently attached to C5, instead of the normal N1. Each rRNA can contain up to 100 pseudouridine ('psi') residues, which may serve to stabilize the conformation of rRNAs.
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Product Notes
The Dkc1 dkc1 (Catalog #AAA961082) is a Recombinant Protein produced from E Coli or Yeast or Baculovirus or Mammalian Cell and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is 2-509, Full length protein. The amino acid sequence is listed below: ADAEAAMTFP KKHKKKKERK PLPEADVAEI QHAEDFLIKP ESKAAQLDTS QWPLLLKNFD RLNVRTTHYT PIPCGSNPLK REIGEYVRTG FINLDKPSNP SSHEVVAWIR RILRVEKTGH SGTLDPKVTG CLIVCIERAT RLVKSQQSAG KEYVGVVRLH NAIEGTAQLS RALETLTGAL FQRPPLIAAV KRQLRVRTIY ESRVVEYDPE RRLGVFWVSC EAGTYIRTLC VHLGLLLGVG GQMQELRRVR SGVVGERDHM VTMHDVLDAQ YLYDHHRDES YLRRVVFPLE KLLTSHKRLV MKDSAVNAIC YGAKIMLPGL LRYEDGIEVN QEVVVITTKG EAVCVAIALM TTAVISTCDH GVVAKIKRVI MERDTYPRKW GLGPKASQKK QLIKQGLLDK HGRPTDGTPA SWTRDYVDYS DSSKKATAAE ATPGPGVTAD AASIVKRKRD SDSDADEATP TTTPRVKKEK KKKKEKADGG EEAAEDGDGD ATRKKKKKKA RAAEELSG. It is sometimes possible for the material contained within the vial of "H/ACA ribonucleoprotein complex subunit 4 (Dkc1), Recombinant Protein" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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