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

Chromosome: 21; NC_000021.8 (47556065..47575499, complement). Location: 21q22.3

Sf9 insect cells

Highly Purified
95% (SDS-PAGE)

Supplied as a liquid in 16mM HEPES buffer, pH 7.6, 240mM sodium chloride and 20% glycerol.

Store at 4 degree C if entire vial will be used within 2-4 weeks. Store, frozen at -20 degree Cfor longer periods of time. Please avoid freeze-thaw cycles.

Small volumes of FTCD recombinant protein 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.

Formiminotransferase cyclodeaminase, a bifunctional enzyme of tetrahydrofolate synthesis, is the target antigen of anti-LC1 (liver cytosol antigen type 1) autoantibodies. Presence of LC1 autoantibodies is a marker for type 2 autoimmune hepatitis (for which anti-LKM 1/cytochrome P450 2D6 autoantibodies are a further marker). Serum LC1 autoantibody concentrations appear to fluctuate in parallel with aminotransferase levels, a particularly intriguing observation that suggests a possible role of LC1 autoreactivity in the pathogenic mechanism leading to hepatocyte injury.

Recombinant Human Formiminotransferase Cyclodeaminase 1 produced in SF9 is a glycosylated, polypeptide chain having a molecular mass of 59,749 dalton. Recombinant LC-1 is expressed with a -6xHis tag and purified by proprietary chromatographic techniques.

Molecular Biology; MB-Enzymes

Western-Blot with monoclonal anti-hexa-His-tag antibody.and autoimmune hepatitis (AIH II) patient sera.

59.75kD[Similar Products]

formimidoyltransferase cyclodeaminase

formimidoyltransferase-cyclodeaminase; formiminotransferase cyclodeaminase; formiminotransferase-cyclodeaminase

Formimidoyltransferase-cyclodeaminase

FTCD??[Similar Products]

FTCD_HUMAN

The protein encoded by this gene is a bifunctional enzyme that channels 1-carbon units from formiminoglutamate, a metabolite of the histidine degradation pathway, to the folate pool. Mutations in this gene are associated with glutamate formiminotransferase deficiency. Alternatively spliced transcript variants have been found for this gene.[provided by RefSeq, Dec 2009]

FTCD: Folate-dependent enzyme, that displays both transferase and deaminase activity. Serves to channel one-carbon units from formiminoglutamate to the folate pool. Defects in FTCD are the cause of glutamate formiminotransferase deficiency (FIGLU-URIA); also known as formiminoglutamicaciduria (FIGLU-uria). It is an autosomal recessive disorder. Features of a severe phenotype, include elevated levels of formiminoglutamate (FIGLU) in the urine in response to histidine administration, megaloblastic anemia, and mental retardation. Features of a mild phenotype include high urinary excretion of FIGLU in the absence of histidine administration, mild developmental delay, and no hematological abnormalities. 4 isoforms of the human protein are produced by alternative splicing.

Protein type: Cofactor and Vitamin Metabolism - one carbon pool by folate; EC 2.1.2.5; Lyase; EC 4.3.1.4; Methyltransferase; Amino Acid Metabolism - histidine

Chromosomal Location of Human Ortholog: 21q22.3

Cellular Component: centriole; Golgi apparatus; cytoplasm; cytosol

Molecular Function: formimidoyltetrahydrofolate cyclodeaminase activity; glutamate formimidoyltransferase activity; folic acid binding

Biological Process: folic acid and derivative metabolic process; histidine catabolic process to glutamate and formamide; cytoskeleton organization and biogenesis; histidine catabolic process; histidine catabolic process to glutamate and formate

Disease: Formiminotransferase Deficiency

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