catalog number :
MBS204299
products type :
Recombinant Protein
products full name :
Mimitin, 1-169aa, Human, His tag, E Coli
products short name :
Mimitin
products name syn :
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2; Mimitin; MMTN; NDuFA12L
other names :
mimitin, mitochondrial; Mimitin, mitochondrial; mimitin, mitochondrial; Myc-induced mitochondrial protein; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, assembly factor 2; NDUFA12-like protein; NADH dehydrogenase (ubiquinone) complex I, assembly factor 2; B17.2-like; B17.2L; Myc-induced mitochondrial protein; MMTN; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2; NDUFA12-like protein
other gene names :
NDUFAF2; NDUFAF2; MMTN; B17.2L; mimitin; NDUFA12L; NDUFA12L; B17.2L; MMTN
uniprot entry name :
MIMIT_HUMAN
sequence :
MGSSHHHHHH SSGLVPRGSH MGWSQDLFRA LWRSLSREVK EHVGTDQFGN KYYYIPQYKN WRGQTIREKR IVEAANKKEV DYEAGDIPTE WEAWIRRTRK TPPTMEEILK NEKHREEIKI KSQDFYEKEK LLSKETSEEL LPPPVQTQIK GHASAPYFGK EEPSVAPSST GKTFQPGSWM PRDGKSHNQ
purity :
> 85% by SDS - PAGE
form :
Liquid. In 20mM Tris-HCl buffer (pH 8.0) containing 1mM DTT, 20% glycerol, 200mM NaCl
concentration :
0.5mg/ml (determined by Bradford assay)
storage stability :
Can be stored at 4 degree C short term (1-2 weeks). For long term storage, aliquot and store at -20 degree C or -70 degree C. Avoid repeated freezing and thawing cycles.
tested application :
SDS-PAGE
other info1 :
Antigen Species: Human. Tag: His-tag
other info2 :
Expression System: E Coli
products categories :
Enzymes & Proteases
products description :
NDuFAF2, also known as Mimitin, belongs to the complex I NDuFA12 subunit family. NADH dehydrogenase is an enzyme located in the inner mitochondrial membrane that catalyzes the transfer of electrons from NADH to coenzyme Q (CoQ). It is the 'entry enzyme' of oxidative phosphorylation in the mitochondria. Mimitin protein acts as a molecular chaperone for mitochondrial complex I assembly. Recombinant human NDuFAF2 protein, fused to His-tag at N-terminus, was expressed in E.coli and purified by using conventional chromatography techniques.
products references :
Tsuneoka M., et al (2005) J. Biol. Chem. 280:19977-19985. Ogilvie I., et al (2005) J. Clin. Invest. 115:2784-2792
ncbi gb acc num :
NM_174889.4
ncbi mol weight :
22kDa (189aa), confirmed by MALDI-TOF
ncbi pathways :
Validated Targets Of C-MYC Transcriptional Activation Pathway (169351)
ncbi summary :
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 summary :
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 adult-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