Rabbit anti-Human NDUFAF2 Polyclonal Antibody | anti-NDUFAF2 antibody

NDUFAF2, ID (NDUFAF2, NDUFA12L, Mimitin, mitochondrial, B17.2-like, Myc-induced mitochondrial protein, NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2, NDUFA12-like protein) (MaxLight 650)

Cat. Num. AAA6324405

• Gene Names: NDUFAF2; MMTN; B17.2L; mimitin; NDUFA12L
• Reactivity: Human
• Applications: Immunohistochemistry, Western Blot
• Purity: Purified by Protein A and Peptide Affinity Chromatography.
• Synonyms: NDUFAF2; Polyclonal Antibody; ID (NDUFAF2; NDUFA12L; Mimitin; mitochondrial; B17.2-like; Myc-induced mitochondrial protein; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2; NDUFA12-like protein) (MaxLight 650); NDUFA12-like protein; anti-NDUFAF2 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human NDUFAF2.
• Purity/Purification: Purified by Protein A and Peptide Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with MaxLight650.

• Applicable Applications for anti-NDUFAF2 antibody: Immunohistochemistry (IHC) Paraffin, Western Blot (WB), FLISA
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 71-99 in the central terminal region of human NDUFAF2.
• Conjugate: MaxLight650
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: MaxLight650 conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western Blot analysis in HepG2 cell line lysates (35ug/lane), usingMBS649912. This demonstrates that MBS649912 detected the NDUFAF2 protein (arrow).)

Immunohistochemistry (IHC)

(Immunohistochemistry analysis in formalin fixed and paraffin embedded human skeletal muscle, using MBS649912 followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of MBS649912 for immunohistochemistry.)

Product Categories/Family for anti-NDUFAF2 antibody

Antibodies; Mitochondrial Proteins

References

1. Rose, J.E., et al. Mol. Med. 16 (7-8), 247-253 (2010). 2. Lesch, K.P., et al. Mol. Psychiatry (2010). 3. Herzer, M., et al. Neuropediatrics 41(1):30-34(2010). 4. Hoefs, S.J., et al. Hum. Mutat. 30 (7), E728-E736 (2009). 5. Wang, L., et al. Cancer Epidemiol. Biomarkers Prev. 17(12):3558-3566(2008).

NCBI and Uniprot Product Information

• NCBI GI #: 29789409
• NCBI GeneID: 91942
• NCBI Accession #: NP_777549.1
• NCBI GenBank Nucleotide #: NM_174889.4
• UniProt Accession #: Q8N183; A8K5I1
• 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
• NCBI Official Synonym Symbols: MMTN; B17.2L; mimitin; NDUFA12L
• 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
• Protein Family: Mimitin
• UniProt Gene Name: NDUFAF2
• UniProt Synonym Gene Names: NDUFA12L; B17.2L; MMTN
• UniProt Entry Name: MIMIT_HUMAN

NCBI Description

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 Description

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