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Rabbit Anti-NDUFAF1 Recombinant Antibody (CBWJN-1259) (CBMAB-N1626-WJ)

This product is a Rabbit antibody that recognizes NDUFAF1. The antibody CBWJN-1259 can be used for immunoassay techniques such as: WB, IP, ICC, IHC-P.
See all NDUFAF1 antibodies

Summary

Host Animal
Rabbit
Specificity
Mouse, Rat, Human
Clone
CBWJN-1259
Antibody Isotype
IgG
Application
WB, IP, ICC, IHC-P

Basic Information

Specificity
Mouse, Rat, Human
Antibody Isotype
IgG
Clonality
Monoclonal
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
Buffer
PBS, 49%, Glycerol 50%, BSA 0.05%
Preservative
0.01% Sodium azide
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 1
Introduction
This gene encodes a complex I assembly factor protein. Complex I (NADH-ubiquinone oxidoreductase) 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. The encoded protein is required for assembly of complex I, and mutations in this gene are a cause of mitochondrial complex I deficiency. Alternatively spliced transcript variants have been observed for this gene, and a pseudogene of this gene is located on the long arm of chromosome 19. [provided by RefSeq, Dec 2011]
Entrez Gene ID
Human51103
Mouse69702
Rat296086
UniProt ID
HumanQ9Y375
MouseQ9CWX2
RatF1LWG4
Alternative Names
NADH:Ubiquinone Oxidoreductase Complex Assembly Factor 1; NADH Dehydrogenase (Ubiquinone) Complex I, Assembly Factor 1; CIA30; NADH Dehydrogenase (Ubiquinone) 1 Alpha Subcomplex, Assembly Factor 1; NADH-Ubiquinone Oxidoreductase 1 Alpha Subcomplex, Assembly Factor 1; NADH Dehydrogenase [Ubiquinone] 1 Alpha Subcomplex Assembly Factor 1; Complex I Intermediate-Associated Protein 30, Mitochondrial; CGI-65; CGI65;
Function
As part of the MCIA complex, involved in the assembly of the mitochondrial complex I.
Biological Process
Chaperone-mediated protein complex assembly Source: UniProtKB
Mitochondrial electron transport, NADH to ubiquinone Source: GO_Central
Mitochondrial respiratory chain complex I assembly Source: UniProtKB
NADH dehydrogenase complex assembly Source: GO_Central
Protein-containing complex assembly Source: UniProtKB
Cellular Location
Mitochondrion
Mitochondrion matrix
Note: Peripherally associated with the matrix face of the mitochondrial inner membrane.
Involvement in disease
Mitochondrial complex I deficiency, nuclear type 11 (MC1DN11):
A form of mitochondrial complex I deficiency, the most common biochemical signature of mitochondrial disorders, a group of highly heterogeneous conditions characterized by defective oxidative phosphorylation, which collectively affects 1 in 5-10000 live births. Clinical disorders have variable severity, ranging 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. MC1DN11 transmission pattern is consistent with autosomal recessive inheritance.
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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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