Human NHP2 Recombinant Protein (RPPB4095)

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SKU:
RPPB4095
Product type:
Recombinant Protein
Size:
20ug
Species:
Human
Target:
NHP2
Synonyms:
H/ACA ribonucleoprotein complex subunit 2
Synonyms:
Nucleolar protein family A member 2
Synonyms:
snoRNP protein NHP2
Synonyms:
NHP2
Source:
Escherichia Coli
Uniprot:
Q9NX24
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Description

Product Name:Human NHP2 Recombinant Protein
Product Code:RPPB4095
Size:20µg
Species:Human
Target:NHP2
Synonyms:H/ACA ribonucleoprotein complex subunit 2, Nucleolar protein family A member 2, snoRNP protein NHP2, NHP2, NOLA2, NHP2P.
Source:Escherichia Coli
Physical Appearance:Sterile Filtered colorless solution.
Formulation:The NHP2 solution (0.25 mg/ml) 20mM Tris-HCl buffer (pH8.0), 20% glycerol, 0.1M NaCl and 1mM DTT.
Stability:Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.
Purity:Greater than 90.0% as determined by SDS-PAGE.
Amino Acid Sequence:MGSSHHHHHH SSGLVPRGSH MTKIKADPDG PEAQAEACSG ERTYQELLVN QNPIAQPLAS RRLTRKLYKC IKKAVKQKQI RRGVKEVQKF VNKGEKGIMV LAGDTLPIEV YCHLPVMCED RNLPYVYIPS KTDLGAAAGS KRPTCVIMVK PHEEYQEAYD ECLEEVQSLP LPL

NHP2 belongs to the H/ACA snoRNPs (small nucleolar ribonucleoproteins) gene family. snoRNPs are involved in various aspects of rRNA processing and modification and have been organized into 2 families: C/D and H/ACA. NHP2 (NOLA2) forms a small ribonucleoprotein particle with GAR1 (NOLA1), Nop10 for the isomerization of uridine to pseudouridine. NHP2 is involved in a variety of aspects of rRNA processing and modification. NHP2 localizes to the dense fibrillar component of the nucleolus and in nuclear Cajal bodies.

NHP2 Human Recombinant fused with a 20 amino acid His tag at N-terminus produced in E.Coli is a single, non-glycosylated, polypeptide chain containing 173 amino acids (1-153 a.a.) and having a molecular mass of 19.3kDa. The NHP2 is purified by proprietary chromatographic techniques.

UniProt Protein Function: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.
UniProt Protein Details:

Protein type:Nucleolus

Chromosomal Location of Human Ortholog: 5q35.3

Cellular Component: nucleoplasm; Cajal body; small nucleolar ribonucleoprotein complex; cytoplasm; nucleolus

Molecular Function:snoRNA binding

Biological Process: rRNA pseudouridine synthesis

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

NCBI Summary: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]
UniProt Code:Q9NX24
NCBI GenInfo Identifier:68565945
NCBI Gene ID:55651
NCBI Accession:Q9NX24.1
UniProt Secondary Accession:Q9NX24,Q9P095, A6NKY8,
UniProt Related Accession:Q9NX24
Molecular Weight:153
NCBI Full Name:H/ACA ribonucleoprotein complex subunit 2
NCBI Synonym Full Names:NHP2 ribonucleoprotein
NCBI Official Symbol:NHP2  
NCBI Official Synonym Symbols:DKCB2; NHP2P; NOLA2  
NCBI Protein Information:H/ACA ribonucleoprotein complex subunit 2; NHP2-like protein; snoRNP protein NHP2; NHP2 ribonucleoprotein homolog; nucleolar protein family A, member 2 (H/ACA small nucleolar RNPs)
UniProt Protein Name:H/ACA ribonucleoprotein complex subunit 2
UniProt Synonym Protein Names:Nucleolar protein family A member 2; snoRNP protein NHP2
Protein Family:NHP2-like protein
UniProt Gene Name:NHP2  
UniProt Entry Name:NHP2_HUMAN
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