Human CNBP Recombinant Protein (RPPB3213)

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RPPB3213
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Product Name:Human CNBP Recombinant Protein
Product Code:RPPB3213
Size:20µg
Species:Human
Target:CNBP
Synonyms:CNBP1, DM2, PROMM, RNF163, ZCCHC22, ZNF9, Cellular nucleic acid-binding protein, CNBP, Zinc finger protein 9.
Source:Escherichia Coli
Physical Appearance:Sterile Filtered colorless solution.
Formulation:CNBP protein solution (0.5mg/ml) containing 20mM Tris-HCl buffer (pH 7.5), 0.2M NaCl, 20% glycerol 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% as determined by SDS-PAGE.
Amino Acid Sequence:MGSSHHHHHH SSGLVPRGSH MGSMSSNECF KCGRSGHWAR ECPTGGGRGR GMRSRGRGFQ FVSSSLPDIC YRCGESGHLA KDCDLQEDAC YNCGRGGHIA KDCKEPKRER EQCCYNCGKP GHLARDCDHA DEQKCYSCGE FGHIQKDCTK VKCYRCGETG HVAINCSKTS EVNCYRCGES GHLARECTIE ATA

Cellular nucleic acid-binding protein (CNBP) is a nucleic-acid binding protein with seven zinc-finger domains, which has a preference for binding single stranded DNA and RNA. CNBP serves in cap-independent translation of ornithine decarboxylase mRNA, and also in sterol-mediated transcriptional regulation. The expansion of A CCTG in CNBP protein�s first intron results in myotonic dystrophy type 2.

CNBP Human Recombinant produced in E.Coli is a single, non-glycosylated polypeptide chain containing 193 amino acids (1-170 a.a) and having a molecular mass of 21kDa. CNBP is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

UniProt Protein Function:ZNF9: Single stranded DNA-binding protein, with specificity to the sterol regulatory element (SRE). Involved in sterol-mediated repression. Defects in CNBP are the cause of dystrophia myotonica type 2 (DM2); also known as proximal myotonic myopathy (PROMM). A multisystem disease characterized by the association of proximal muscle weakness with myotonia, cardiac manifestations and cataract. Additional features can include hyperhidrosis, testicular atrophy, insulin resistance and diabetes and central nervous system anomalies in rare cases. The causative mutation is a CCTG expansion (mean approximately 5000 repeats) located in intron 1 of the CNBP gene. 5 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:

Protein type:RNA-binding; DNA-binding; Transcription factor

Chromosomal Location of Human Ortholog: 3q21

Cellular Component: cytosol; endoplasmic reticulum; nucleus

Molecular Function:protein binding; transcription factor activity

Biological Process: cholesterol biosynthetic process; positive regulation of cell proliferation; positive regulation of transcription from RNA polymerase II promoter; positive regulation of transcription, DNA-dependent; regulation of transcription, DNA-dependent

Disease: Myotonic Dystrophy 2

NCBI Summary:This gene encodes a nucleic-acid binding protein with seven zinc-finger domains. The protein has a preference for binding single stranded DNA and RNA. The protein functions in cap-independent translation of ornithine decarboxylase mRNA, and may also function in sterol-mediated transcriptional regulation. A CCTG expansion from <30 repeats to 75-11000 repeats in the first intron of this gene results in myotonic dystrophy type 2. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2016]
UniProt Code:P62633
NCBI GenInfo Identifier:50401852
NCBI Gene ID:7555
NCBI Accession:P62633.1
UniProt Secondary Accession:P62633,P20694, Q4JGY0, Q4JGY1, Q5QJR0, A8K7V4, B2RAV9 B4DP17, D3DNB9, D3DNC0, D3DNC1, E9PDR7,
UniProt Related Accession:P62633
Molecular Weight:18,871 Da
NCBI Full Name:Cellular nucleic acid-binding protein
NCBI Synonym Full Names:CCHC-type zinc finger nucleic acid binding protein
NCBI Official Symbol:CNBP��
NCBI Official Synonym Symbols:DM2; ZNF9; CNBP1; PROMM; RNF163; ZCCHC22��
NCBI Protein Information:cellular nucleic acid-binding protein
UniProt Protein Name:Cellular nucleic acid-binding protein
UniProt Synonym Protein Names:Zinc finger protein 9
Protein Family:Cellular nucleic acid-binding protein
UniProt Gene Name:CNBP��
UniProt Entry Name:CNBP_HUMAN
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Additional Information

Product Type:
Recombinant Protein
Species:
Human
Uniprot:
P62633
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