PQBP1 Human

Polyglutamine Binding Protein 1 Human Recombinant
Cat. No.
BT8460
Source
E.coli.
Synonyms
Golyglutamine binding protein 1, Polyglutamine tract-binding protein 1, 38kDa nuclear protein containing a WW domain, mental retardation X-linked 55, Sutherland-Haan X-linked mental retardation syndrome, Npw38, MRXS3, MRX55, MRXS8, RENS1, SHS.
Appearance
Sterile Filtered colorless solution.
Purity
Greater than 95% as determined by SDS-PAGE.
Usage
THE BioTek's products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.
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Description

PQBP1 Human Recombinant produced in E. coli is a single polypeptide chain containing 289 amino acids (1-265) and having a molecular mass of 33.0kDa.
PQBP1 is fused to a 24 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

Product Specs

Introduction
PQBP1, a transcription repressor, interacts with polyglutamine tract-containing transcription regulators and genes associated with neurodegenerative disorders. It is primarily located in the nucleus of neurons throughout the brain, exhibiting elevated levels in the hippocampus, olfactory bulb, and cerebellar cortex. PQBP1 possesses a WWP/WW domain for binding proline-rich motifs and a C2 domain capable of mediating Ca2+-dependent phospholipid signaling.
Description
Recombinantly produced in E. coli, PQBP1 Human is a single polypeptide chain comprising 289 amino acids (residues 1-265) with a molecular weight of 33.0 kDa. A 24 amino acid His-tag is fused to the N-terminus of PQBP1, which is purified using proprietary chromatographic techniques.
Physical Appearance
A sterile, colorless solution that has been filtered.
Formulation
The PQBP1 solution is provided at a concentration of 0.5 mg/ml in a buffer consisting of 20 mM Tris-HCl (pH 8.0), 1 mM DTT, 100 mM NaCl, and 20% glycerol.
Stability
For optimal storage, refrigerate the product at 4°C if the entire vial will be used within 2-4 weeks. For extended storage, freeze the product at -20°C. The addition of a carrier protein (0.1% HSA or BSA) is recommended for long-term storage. Repeated freezing and thawing should be avoided.
Purity
SDS-PAGE analysis indicates a purity greater than 95%.
Synonyms
Golyglutamine binding protein 1, Polyglutamine tract-binding protein 1, 38kDa nuclear protein containing a WW domain, mental retardation X-linked 55, Sutherland-Haan X-linked mental retardation syndrome, Npw38, MRXS3, MRX55, MRXS8, RENS1, SHS.
Source
E.coli.
Amino Acid Sequence
MGSSHHHHHH SSGLVPRGSH MGSHMPLPVA LQTRLAKRGI LKHLEPEPEE EIIAEDYDDD PVDYEATRLE GLPPSWYKVF DPSCGLPYYW NADTDLVSWL SPHDPNSVVT KSAKKLRSSN ADAEEKLDRS HDKSDRGHDK SDRSHEKLDR GHDKSDRGHD KSDRDRERGY DKVDRERERD RERDRDRGYD KADREEGKER RHHRREELAP YPKSKKAVSR KDEELDPMDP SSYSDAPRGT WSTGLPKRNE AKTGADTTAA GPLFQQRPYP SPGAVLRANA EASRTKQQD

Product Science Overview

Gene and Protein Structure

The PQBP1 gene is located on the X chromosome (Xp11.23) and encodes a nuclear polyglutamine-binding protein that contains a WW domain . This domain is crucial for protein-protein interactions, particularly with polyglutamine tracts found in certain transcription factors and disease-related proteins .

Function and Mechanism

PQBP1 acts as a scaffold protein, interacting with various splicing-related factors and regulating alternative splicing of target pre-mRNA species . It also plays a role in transcription regulation by interacting with the transcription machinery and can either activate or suppress transcription depending on the context . Additionally, PQBP1 is involved in the innate immune response and neuron development .

Clinical Significance

Mutations in the PQBP1 gene have been associated with several X-linked cognitive disabilities, including Renpenning syndrome 1 . These mutations can lead to various developmental and neurological issues, highlighting the importance of PQBP1 in normal brain function and development .

Research and Applications

Research on PQBP1 has provided insights into its role in cellular processes and its potential involvement in neurodegenerative diseases. The recombinant form of PQBP1 is used in various studies to understand its interactions and functions better . This research is crucial for developing potential therapeutic strategies for conditions associated with PQBP1 mutations .

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