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Mouse Anti-NDUFAF7 Recombinant Antibody (CBFYM-3027) (CBMAB-M3222-FY)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
CBFYM-3027
Antibody Isotype
IgG2b
Application
WB, IHC, IHC-P

Basic Information

Immunogen
Full length human recombinant protein of human C2orf56(NP_653337) produced in HEK293T cell
Specificity
Human
Antibody Isotype
IgG2b
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
NADH:Ubiquinone Oxidoreductase Complex Assembly Factor 7
Introduction
This gene encodes an assembly factor protein which helps in the assembly and stabilization of Complex I, a large multi-subunit enzyme in the mitochondrial respiratory chain. Complex I is involved in several physiological activities in the cell, including metabolite transport and ATP synthesis. The encoded protein is a methyltransferase which methylates Arg85 of a subunit of Complex I in the early stages of its assembly. A pseudogene related to this gene is located on chromosome 8. Alternative splicing results in multiple transcript variants.
Entrez Gene ID
55471
UniProt ID
Q7L592
Alternative Names
NADH:Ubiquinone Oxidoreductase Complex Assembly Factor 7; NADH Dehydrogenase (Ubiquinone) Complex I, Assembly Factor 7; NADH Dehydrogenase [Ubiquinone] Complex I, Assembly Factor 7; Mitochondrial Dysfunction Protein A Homolog; C2orf56; Protein Arginine Methyltransferase NDUFAF7, Mitochondrial; Protein MidA Homolog, Mitochondrial
Function
Arginine methyltransferase involved in the assembly or stability of mitochondrial NADH:ubiquinone oxidoreductase complex (complex I) (PubMed:20406883, PubMed:24089531, PubMed:24838397).

Acts by mediating symmetric dimethylation of 'Arg-118' of NDUFS2 after it assembles into the complex I, stabilizing the early intermediate complex (PubMed:24089531).
Biological Process
Mitochondrial respiratory chain complex I assembly Source: UniProtKB
Peptidyl-arginine methylation, to symmetrical-dimethyl arginine Source: UniProtKB
Cellular Location
Mitochondrion
Involvement in disease
Defects in NDUFAF7 may be a cause of susceptibility to pathologic myopia, a genetically heterogeneous disorder characterized by extreme, familial, early-onset vision loss and described as myopia accompanied by severe deformation of the eye besides excessive elongation of the eye.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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