FOXRED1, Polyclonal Antibody

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

Affinity purified

PBS, pH 7.4 with 0.02% sodium azide.

1.0 mg/ml (lot specific)

This product is stable for several weeks at 4 degree C as an undiluted liquid. Dilute only prior to immediate use. For extended storage, aliquot contents and freeze at -20 degree C or below. Avoid cycles of freezing and thawing. Expiration date is one (1) year from date of receipt.

Small volumes of anti-FOXRED1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Total protein Ab

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

ELISA: 1:20000-1:80000
WB: 1:200-1:1000
IHC: 1:100-1:500

52,104 Da

FAD-dependent oxidoreductase domain containing 1

FAD-dependent oxidoreductase domain-containing protein 1

FAD-dependent oxidoreductase domain-containing protein 1

FXRD1_HUMAN

This gene encodes a protein that contains a FAD-dependent oxidoreductase domain. The encoded protein is localized to the mitochondria and may function as a chaperone protein required for the function of mitochondrial complex I. Mutations in this gene are associated with mitochondrial complex I deficiency. Alternatively spliced transcript variants have been observed for this gene. [provided by RefSeq, Dec 2010]

FOXRED1: Defects in FOXRED1 are a cause of mitochondrial complex I deficiency (MT-C1D). A disorder of the mitochondrial respiratory chain that causes a wide range of clinical manifestations from lethal neonatal disease to *****-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. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: Mitochondrial; Membrane protein, integral

Chromosomal Location of Human Ortholog: 11q24.2

Cellular Component: mitochondrion; integral to membrane

Molecular Function: oxidoreductase activity

Disease: Leigh Syndrome; Mitochondrial Complex I Deficiency

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