FECH, Polyclonal Antibody

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

Polyclonal

IgG

Rabbit

>=95% as determined by SDS-PAGE
Immunogen Affinity Purified

Liquid

PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Small volumes of anti-FECH 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.

ELISA (EIA), Western Blot (WB)

48,625 Da

ferrochelatase

ferrochelatase, mitochondrial

Ferrochelatase, mitochondrial

HEMH_HUMAN

The protein encoded by this gene is localized to the mitochondrion, where it catalyzes the insertion of the ferrous form of iron into protoporphyrin IX in the heme synthesis pathway. Mutations in this gene are associated with erythropoietic protoporphyria. Two transcript variants encoding different isoforms have been found for this gene. A pseudogene of this gene is found on chromosome 3.[provided by RefSeq, May 2010]

FECH: Catalyzes the ferrous insertion into protoporphyrin IX. Defects in FECH are the cause of erythropoietic protoporphyria (EPP). Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. EPP is a form of porphyria marked by excessive protoporphyrin in erythrocytes, plasma, liver and feces, and by widely varying photosensitive skin changes ranging from a burning or pruritic sensation to erythema, edema and wheals. Belongs to the ferrochelatase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Mitochondrial; EC 4.99.1.1; Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; Lyase

Chromosomal Location of Human Ortholog: 18q21.3

Cellular Component: mitochondrial inner membrane; mitochondrial matrix; mitochondrion

Molecular Function: 2 iron, 2 sulfur cluster binding; ferrochelatase activity; ferrous iron binding; protein binding

Biological Process: generation of precursor metabolites and energy; heme biosynthetic process; protoporphyrinogen IX metabolic process; response to arsenic; response to drug; response to ethanol; response to insecticide; response to lead ion; response to light stimulus; response to methylmercury

Disease: Protoporphyria, Erythropoietic

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