NOLA2; H/ACA ribonucleoprotein complex subunit 2; Nucleolar protein family A member 2; snoRNP protein NHP2

For Research Use Only. Not for use in diagnostic procedures.

E Coli

Greater than 95% as determined by reducing SDS-PAGE.

Supplied as a 0.2 muM filtered solution of 20mM Tris, 100mM NaCl, 1mM DTT, pH 8.0.

Lyophilized protein should be stored at -20 degree C, though stable at room temperature for 3 weeks. Reconstituted protein solution can be stored at 2-8 degree C for 2-7 days. Aliquots of reconstituted samples are stable at -20 degree C for 3 months.

Small volumes of NHP2 recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Recombinant Human NHP2 Protein is produced by our E Coli expression system and the target gene encoding Met1-Leu153 is expressed with a 6His tag at the N-terminus.

ELISA (EIA),Western Blot (WB), SDS-PAGE, Mass Spectrometry (MS)

17,201 Da

NHP2 ribonucleoprotein

H/ACA ribonucleoprotein complex subunit 2

H/ACA ribonucleoprotein complex subunit 2

NOLA2??[Similar Products]

NHP2_HUMAN

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 DKC1, NOLA1 and NOLA3 proteins. These four H/ACA snoRNP 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. The four H/ACA snoRNP proteins are also components of the telomerase complex. This gene encodes a protein related to Saccharomyces cerevisiae Nhp2p. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Oct 2008]

NOLA2: Required for ribosome biogenesis and telomere maintenance. Part of the 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. May also be required for correct processing or intranuclear trafficking of TERC, the RNA component of the telomerase reverse transcriptase (TERT) holoenzyme. Defects in NHP2 are the cause of dyskeratosis congenita autosomal recessive type 2 (DKCB2). A rare multisystem disorder caused by defective telomere maintenance. It is characterized by progressive bone marrow failure, and the clinical triad of reticulated skin hyperpigmentation, nail dystrophy, and mucosal leukoplakia. Common but variable features include premature graying, aplastic anemia, low platelets, osteoporosis, pulmonary fibrosis, and liver fibrosis among others. Early mortality is often associated with bone marrow failure, infections, fatal pulmonary complications, or malignancy. Belongs to the ribosomal protein L7Ae family.

Protein type: Nucleolus

Chromosomal Location of Human Ortholog: 5q35.3

Cellular Component: cytoplasm; nuclear chromosome, telomeric region; nucleoplasm; small nucleolar ribonucleoprotein complex; telomerase holoenzyme complex

Molecular Function: protein binding; snoRNA binding

Biological Process: cleavages during rRNA processing; maturation of LSU-rRNA; rRNA pseudouridine synthesis; snRNA pseudouridine synthesis; telomere maintenance via telomerase; translation

Disease: Dyskeratosis Congenita, Autosomal Recessive, 1; Dyskeratosis Congenita, Autosomal Recessive, 2

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