NDUFAF1 Human

NADH Dehydrogenase 1 Alpha Subcomplex, Assembly Factor 1 Human Recombinant
Cat. No.
BT12151
Source
Escherichia Coli.
Synonyms
Complex I intermediate-associated protein 30, mitochondrial, NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 1, NDUFAF1, CIA30, CGI-65, CGI65.
Appearance
Sterile Filtered colorless solution.
Purity
Greater than 90.0% 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

NDUFAF1 Human Recombinant produced in E.coli is a single, non-glycosylated polypeptide chain containing 326 amino acids (25-327) and having a molecular mass of 37kDa.
NDUFAF1 is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

Product Specs

Introduction
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4 (NDUFAF4) plays a crucial role in assembling the mitochondrial NADH: ubiquinone oxidoreductase complex, also known as complex I. This protein is also involved in regulating cell proliferation and survival in hormone-dependent tumor cells. Additionally, NDUFAF4 may be implicated in the regulation of breast tumor cell invasion. Mutations in the NDUFAF4 gene are known to cause mitochondrial complex I deficiency.
Description
Recombinant human NDUFAF1, expressed in E. coli, is a single, non-glycosylated polypeptide chain. It consists of 326 amino acids (residues 25-327) and has a molecular weight of 37 kDa. The protein includes a 23 amino acid His-tag fused to its N-terminus and is purified using proprietary chromatographic techniques.
Physical Appearance
Clear, colorless, and sterile-filtered solution.
Formulation
The NDUFAF1 solution is provided at a concentration of 0.5 mg/ml. The solution is buffered with 20mM Tris-HCl at pH 8.0 and contains 0.15M NaCl, 10% glycerol, and 1mM DTT.
Stability
For short-term storage (2-4 weeks), the solution should be kept at 4°C. For extended storage, it is recommended to freeze the solution at -20°C. To further enhance long-term stability, consider adding a carrier protein such as HSA or BSA (0.1%). It is crucial to avoid repeated freeze-thaw cycles to maintain protein integrity.
Purity
The purity of NDUFAF1 is determined by SDS-PAGE analysis and is guaranteed to be greater than 90%.
Synonyms
Complex I intermediate-associated protein 30, mitochondrial, NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 1, NDUFAF1, CIA30, CGI-65, CGI65.
Source
Escherichia Coli.
Amino Acid Sequence

MGSSHHHHHH SSGLVPRGSH MGSYPFLGIR FAEYSSSLQK PVASPGKASS QRKTEGDLQG DHQKEVALDI TSSEEKPDVS FDKAIRDEAI YHFRLLKDEI VDHWRGPEGH PLHEVLLEQA KVVWQFRGKE DLDKWTVTSD KTIGGRSEVF LKMGKNNQSA LLYGTLSSEA PQDGESTRSG YCAMISRIPR GAFERKMSYD WSQFNTLYLR VRGDGRPWMV NIKEDTDFFQ RTNQMYSYFM FTRGGPYWQE VKIPFSKFFF SNRGRIRDVQ HELPLDKISS IGFTLADKVD GPFFLEIDFI GVFTDPAHTE EFAYENSPEL NPRLFK.

Product Science Overview

Introduction

NADH Dehydrogenase 1 Alpha Subcomplex, Assembly Factor 1 (NDUFAF1) is a crucial protein involved in the assembly of Complex I of the mitochondrial respiratory chain. This complex, also known as NADH:ubiquinone oxidoreductase, is the largest of the five complexes in the electron transport chain and plays a pivotal role in cellular respiration and energy production.

Gene and Protein Structure

The NDUFAF1 gene is located on chromosome 15q15.1 in humans . It encodes a protein that is composed of 327 amino acids and contains an N-terminal mitochondrial targeting sequence . The protein is moderately conserved among species, with the highest homology observed in the C-terminal region .

Function

NDUFAF1 acts as an assembly factor for Complex I, facilitating the correct assembly of this complex within the mitochondrial inner membrane . Complex I catalyzes the transfer of electrons from NADH to ubiquinone (coenzyme Q), which is the first step in the mitochondrial respiratory chain . This process results in the translocation of protons across the inner mitochondrial membrane, contributing to the proton gradient that drives ATP synthesis.

Biological Significance

The proper assembly and function of Complex I are essential for efficient cellular respiration and energy production. Mutations in the NDUFAF1 gene can lead to mitochondrial Complex I deficiency, a condition associated with a range of clinical manifestations, including neurodegenerative diseases and metabolic disorders .

Research and Clinical Implications

Understanding the role of NDUFAF1 in the assembly of Complex I has significant implications for research and clinical practice. Studies on NDUFAF1 can provide insights into the mechanisms underlying mitochondrial diseases and potentially lead to the development of therapeutic strategies for conditions associated with Complex I deficiency .

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