TFB1M Human

Transcription Factor B1, Mitochondrial Human Recombinant
Cat. No.
BT13903
Source
Escherichia Coli.
Synonyms

Dimethyladenosine transferase 1 mitochondrial, Mitochondrial 12S rRNA dimethylase 1, Mitochondrial transcription factor B1, h-mtTFB, h-mtTFB1, hTFB1M, mtTFB1, S-adenosylmethionine-6-N'-N'-adenosyl(rRNA) dimethyltransferase 1, TFB1M, CGI-75, CGI75, mtTFB.

Appearance
Sterile Filtered clear solution.
Purity
Greater than 90.0% as determined by SDS-PAGE.
Usage
Prospec's products are furnished for LABORATORY RESEARCH USE ONLY. They may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.
Shipped with Ice Packs
In Stock

Description

TFB1M Human Recombinant produced in E.Coli is a single, non-glycosylated polypeptide chain containing 340 amino acids (28-346 a.a.) and having a molecular mass of 38.8kDa.
TFB1M is fused to a 21 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

Product Specs

Introduction

As a member of the methyltransferase superfamily, TFB1M functions as a dimethyltransferase that specifically methylates mitochondrial 12S rRNA at its conserved stem loop. This protein is an integral component of the basal mitochondrial transcription complex and plays a critical role in mitochondrial gene expression. Importantly, TFB1M exhibits the ability to stimulate transcription independent of its methyltransferase activity.

Description

Recombinant TFB1M Human, produced in E. coli, is a single, non-glycosylated polypeptide chain consisting of 340 amino acids (28-346 a.a.). With a molecular weight of 38.8 kDa, it features a 21 amino acid His-tag fused at the N-terminus. Purification is achieved through proprietary chromatographic techniques.

Physical Appearance
A clear solution that has undergone sterile filtration.
Formulation

The TFB1M protein solution is provided at a concentration of 0.5 mg/ml. It is formulated in a buffer containing 20 mM Tris-HCl (pH 8.0), 20% glycerol, 0.1 M NaCl, and 1 mM DTT.

Stability

For short-term storage (2-4 weeks), the product should be kept at 4°C. For extended storage, freezing at -20°C is recommended. To ensure optimal stability during long-term storage, the addition of a carrier protein (0.1% HSA or BSA) is advised. It is important to avoid repeated freeze-thaw cycles to maintain product integrity.

Purity
The purity of the TFB1M protein is determined to be greater than 90.0% based on SDS-PAGE analysis.
Synonyms

Dimethyladenosine transferase 1 mitochondrial, Mitochondrial 12S rRNA dimethylase 1, Mitochondrial transcription factor B1, h-mtTFB, h-mtTFB1, hTFB1M, mtTFB1, S-adenosylmethionine-6-N'-N'-adenosyl(rRNA) dimethyltransferase 1, TFB1M, CGI-75, CGI75, mtTFB.

Source
Escherichia Coli.
Amino Acid Sequence

MGSSHHHHHH SSGLVPRGSH MQAAKQLSQN FLLDLRLTDK IVRKAGNLTN AYVYEVGPGP GGITRSILNA DVAELLVVEK DTRFIPGLQM LSDAAPGKLR IVHGDVLTFK VEKAFSESLK RPWEDDPPNV HIIGNLPFSV STPLIIKWLE NISCRDGPFV YGRTQMTLTF QKEVAERLAA NTGSKQRSRL SVMAQYLCNV RHIFTIPGQA FVPKPEVDVG VVHFTPLIQP KIEQPFKLVE KVVQNVFQFR RKYCHRGLRM LFPEAQRLES TGRLLELADI DPTLRPRQLS ISHFKSLCDV YRKMCDEDPQ LFAYNFREEL KRRKSKNEEK EEDDAENYRL.

Product Science Overview

Gene and Protein Structure

TFB1M is encoded by the TFB1M gene, which is located on chromosome 6q25.3 . The protein consists of 346 amino acids and shares homology with bacterial rRNA dimethyltransferases . This homology suggests that TFB1M has evolved from an RNA-modifying enzyme, which is reflected in its dual functionality.

Function and Mechanism

TFB1M is a part of the basal mitochondrial transcription complex. It works in conjunction with mitochondrial RNA polymerase (POLRMT) and mitochondrial transcription factor A (TFAM) to initiate and regulate the transcription of mtDNA . TFB1M binds to the mitochondrial light strand promoter (LSP) and enhances transcription from LSP by TFAM .

In addition to its role in transcription, TFB1M also functions as a dimethyltransferase. It methylates the conserved stem loop of mitochondrial 12S rRNA, which is necessary for the assembly or stability of the small subunit of the mitochondrial ribosome . This methylation is crucial for normal mitochondrial translation, metabolism, and cell growth .

Expression and Localization

TFB1M is ubiquitously expressed in human tissues and localizes to the mitochondria . When transfected into HeLa cells, fluorescence-labeled TFB1M colocalizes with mitochondrial markers, indicating its specific localization within the mitochondria .

Clinical Significance

Mutations or dysregulation of TFB1M can lead to mitochondrial dysfunction, which is associated with various human diseases. For example, TFB1M has been linked to Bardet-Biedl Syndrome 18 and progressive leukoencephalopathy with ovarian failure . Understanding the function and regulation of TFB1M is therefore critical for developing therapeutic interventions for mitochondrial-related diseases.

Research and Therapeutic Potential

Research on TFB1M continues to uncover its multifaceted roles in mitochondrial biology. The protein’s ability to act both as a transcription factor and an rRNA modification enzyme makes it a unique target for therapeutic interventions aimed at correcting mitochondrial dysfunctions .

In conclusion, Transcription Factor B1, Mitochondrial (Human Recombinant) is a vital component of the mitochondrial transcription machinery. Its dual role in transcription and rRNA modification underscores its importance in maintaining mitochondrial function and cellular energy metabolism.

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