FAD-dependent oxidoreductase domain-containing protein 1; 1---; FOXRED1

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

Polyclonal

Rabbit Ig

Rabbit

This FXRD1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 347-377 amino acids from the C-terminal region of human FXRD1.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.5 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

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.

Metabolism; Signal Transduction

Western Blot (WB), ELISA (EIA)

WB~~1:1000

Western blot analysis of FXRD1 Antibody (C-term) in NCI-H460 cell line lysates (35ug/lane). FXRD1 (arrow) was detected using the purified Pab.

53812

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: Membrane protein, integral; Mitochondrial

Chromosomal Location of Human Ortholog: 11q24.2

Cellular Component: mitochondrion; integral to membrane

Molecular Function: oxidoreductase activity

Disease: Leigh Syndrome; Mitochondrial Complex I Deficiency

Lehner, B., et al. Genome Res. 14(7):1315-1323(2004)
Simpson, J.C., et al. EMBO Rep. 1(3):287-292(2000)

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