Product Name
NADH Dehydrogenase 1 Alpha Subcomplex (NDUFAF2), Recombinant Protein
Full Product Name
Recombinant Human NADH Dehydrogenase 1 Alpha Subcomplex, Assembly Factor 2
Product Synonym Names
NDUFAF2 Human; NADH Dehydrogenase 1 Alpha Subcomplex, Assembly Factor 2 Human Recombinant; Mimitin mitochondrial; B17.2-like; B17.2L; Myc-induced mitochondrial protein; MMTN; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2; NDUFA12-like protein; NDUFAF2; NDUFA12L; mimitin
Product Gene Name
NDUFAF2 recombinant protein
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Sequence
MGSSHHH HHH SSGLVPRGSH MGWSQDLFRA LWRSLSREVK EHVGTDQFGN KYYYIPQYKN WRGQTIREKR IVEAANKKEV DYEAGDIPTE WEAWIRRTRK TPPTMEEILK NEKHREEIKI KSQDFYEKEK LLSKETSEEL LPPPVQTQIK GHASAPYFGK EEPSVAPSST GKTFQPGSWM PRDGKSHNQ
3D Structure
ModBase 3D Structure for Q8N183
Purity/Purification
Greater than 85.0% as determined by SDS-PAGE.
Form/Format
NDUFAF2 protein solution (0.5mg/ml) containing 20mM Tris-HCl buffer (pH 8.0), 1mM DTT, 20% glycerol and 200mM NaCl.
Sterile Filtered colorless solution.
Other Notes
Small volumes of NDUFAF2 recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
NDUFAF2 recombinant protein
Description: NDUFAF2 Human Recombinant produced in E Coli is a single, non-glycosylated polypeptide chain containing 189 amino acids (1-169 a.a.) and having a molecular mass of 22kDa.NDUFAF2 is fused to a 20 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.
Introduction: Mimitin (NDUFAF2) is a member of the complex I NDUFA12 subunit family.NADH dehydrogenase is an enzyme located in the inner mitochondrial membrane, which catalyzes the transfer of electrons from NADH to coenzyme Q (CoQ). NDUFAF2 is the "entry enzyme" of oxidative phosphorylation in the mitochondria. Mimitin protein functions as a molecular chaperone for mitochondrial complex I assembly.
Product Categories/Family for NDUFAF2 recombinant protein
ENZYMES; Enzymes; Dehydrogenase
NCBI/Uniprot data below describe general gene information for NDUFAF2. It may not necessarily be applicable to this product.
NCBI Accession #
NP_777549.1
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NCBI GenBank Nucleotide #
NM_174889.4
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UniProt Primary Accession #
Q8N183
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UniProt Secondary Accession #
A8K5I1[Other Products]
UniProt Related Accession #
Q8N183[Other Products]
Molecular Weight
19,856 Da
NCBI Official Full Name
mimitin, mitochondrial
NCBI Official Synonym Full Names
NADH dehydrogenase (ubiquinone) complex I, assembly factor 2
NCBI Official Symbol
NDUFAF2 [Similar Products]
NCBI Official Synonym Symbols
MMTN; B17.2L; mimitin; NDUFA12L
[Similar Products]
NCBI Protein Information
mimitin, mitochondrial; Myc-induced mitochondrial protein; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, assembly factor 2; NDUFA12-like protein
UniProt Protein Name
Mimitin, mitochondrial
UniProt Synonym Protein Names
B17.2-like; B17.2L; Myc-induced mitochondrial protein; MMTN; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2; NDUFA12-like protein
UniProt Gene Name
NDUFAF2 [Similar Products]
UniProt Synonym Gene Names
NDUFA12L; B17.2L; MMTN [Similar Products]
UniProt Entry Name
MIMIT_HUMAN
NCBI Summary for NDUFAF2
NADH:ubiquinone oxidoreductase (complex I) catalyzes the transfer of electrons from NADH to ubiquinone (coenzyme Q) in the first step of the mitochondrial respiratory chain, resulting in the translocation of protons across the inner mitochondrial membrane. This gene encodes a complex I assembly factor. Mutations in this gene cause progressive encephalopathy resulting from mitochondrial complex I deficiency. [provided by RefSeq, Jul 2008]
UniProt Comments for NDUFAF2
NDUFAF2: Acts as a molecular chaperone for mitochondrial complex I assembly. Defects in NDUFAF2 are a cause of mitochondrial complex I deficiency (MT-C1D). A disorder of the mitochondrial respiratory chain that causes a wide range of clinical disorders, from lethal neonatal disease to *****-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease. Belongs to the complex I NDUFA12 subunit family.
Chromosomal Location of Human Ortholog: 5q12.1
Cellular Component: mitochondrion
Molecular Function: NADH dehydrogenase (ubiquinone) activity
Disease: Leigh Syndrome; Mitochondrial Complex I Deficiency
Research Articles on NDUFAF2
1. Within the clinical spectrum of Leigh disease, patients with mutations in NDUFAF2 present with a distinct clinical pattern with predominantly brainstem involvement on MRI
Precautions
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Disclaimer
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