Rabbit Anti-NDUF3/FITC Conjugated antibody

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C3orf60; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3; NDUF3_HUMAN; ndufaf3.

Cat:

SL19067R-FITC

Species Reactivity：

(predicted: Human,Mouse,Rat,Pig,Horse,Rabbit,)

Immunogen：

KLH conjugated synthetic peptide derived from human NDUF3

Format：

Lyophilized or Liquid

Storage instructions：

Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

20kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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background:
This gene encodes a mitochondrial complex I assembly protein that interacts with complex I subunits. Mutations in this gene cause mitochondrial complex I deficiency, a fatal neonatal disorder of the oxidative phosphorylation system. Alternatively spliced transcript variants encoding different isoforms have been identified. [provided by RefSeq, Jul 2009]

Function:
Essential factor for the assembly of mitochondrial NADH:ubiquinone oxidoreductase complex (complex I).

Subunit:
Interacts with NDUFAF4, NDUFS2 and NDUFS3.

Subcellular Location:
Nucleus. Mitochondrion inner membrane.

DISEASE:
Defects in NDUFAF3 are a cause of mitochondrial complex I deficiency (MT-C1D) [MIM:252010]. 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.

Database links:

Entrez Gene: 25915 Human

Entrez Gene: 56769 Rat

Omim: 612911 Human

SwissProt: Q9BU61 Human

SwissProt: O08776 Rat

Unigene: 31387 Human

Unigene: 40118 Rat

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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