Recombinant Human Formiminotransferase Cyclodeaminase | MyBioSource MBS142443 product information

product summary

company name :

MyBioSource

product type :

protein

product name :

Recombinant Human Formiminotransferase Cyclodeaminase

catalog :

MBS142443

quantity :

0.005 mg

price :

140 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS142443

products type :

Recombinant Protein

products full name :

Recombinant Human Formiminotransferase Cyclodeaminase

products short name :

Formiminotransferase Cyclodeaminase

products name syn :

FTCD Human; Formiminotransferase Cyclodeaminase Human Recombinant; Formiminotransferase-cyclodeaminase; FTCD; LCHC1; LC-1

other names :

formimidoyltransferase-cyclodeaminase; Formimidoyltransferase-cyclodeaminase; formimidoyltransferase-cyclodeaminase; formiminotransferase cyclodeaminase; formiminotransferase-cyclodeaminase; formimidoyltransferase cyclodeaminase; Formiminotransferase-cyclodeaminase; FTCD; LCHC1Including the following 2 domains:Glutamate formimidoyltransferase (EC:2.1.2.5)Alternative name(s):Glutamate formiminotransferase; Glutamate formyltransferase

products gene name :

FTCD

other gene names :

FTCD; FTCD; LCHC1; FTCD

uniprot entry name :

FTCD_HUMAN

host :

Sf9 Insect Cells

sequence length :

541

purity :

Greater than 95% as determined by SDS-PAGE.

form :

FTCD is supplied in 16mM HEPES buffer pH-7.6, 240mM sodium chloride, and 20% glycerol.

concentration :

0.35-0.7 ug/ml (depending on the type of ELISA plate and coating buffer).Suitable for biotinylation and iodination.

storage stability :

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. Avoid multiple freeze-thaw cycles.

other info2 :

Immunological Functions: 1. Binds IgG-type human auto-antibodies.2. Standard ELISA test (checker-board analysis of positive/negative sera panels, immuno-dot test).

products categories :

ENZYMES; Enzymes; Deaminase

products description :

Description: Formiminotransferase Cyclodeaminase Human Recombinant (also called liver cytosol type 1) produced in SF9, is a glycosylated, polypeptide chain having a molecular mass of 59,749 Dalton.The FTCD is expressed with a -6xHis tag and purified by proprietary chromatographic techniques. Introduction: Formiminotransferase cyclodeaminase is an enzyme which catalyzes the conversion of formiminoglutamateand tetrahydrofolateinto formiminotetrahydrofolateand glutamate.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.

ncbi gi num :

11140815

ncbi acc num :

NP_006648.1

ncbi gb acc num :

NM_006657.2

uniprot acc num :

O95954

ncbi mol weight :

16,503 Da

ncbi pathways :

Histidine Catabolism Pathway (106186); Histidine Metabolism Pathway (82958); Histidine Metabolism Pathway (324); Metabolic Pathways (132956); Metabolism Pathway (477135); Metabolism Of Amino Acids And Derivatives Pathway (106169); One Carbon Metabolism Pathway (198756); One Carbon Pool By Folate Pathway (83008); One Carbon Pool By Folate Pathway (393); Histidine Degradation Pathway (142356)

ncbi summary :

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]

uniprot summary :

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: Methyltransferase; EC 4.3.1.4; EC 2.1.2.5; Lyase; Cofactor and Vitamin Metabolism - one carbon pool by folate; 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; cytoskeleton organization and biogenesis; histidine catabolic process to glutamate and formamide; histidine catabolic process; histidine catabolic process to glutamate and formate. Disease: Formiminotransferase Deficiency

size1 :

0.005 mg

price1 :

140 USD

size2 :

0.02 mg

price2 :

205

size3 :

1 mg

price3 :

3190

more info or order :

MyBioSource product webpage