FTL; Ferritin L subunit

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

Monoclonal

Mouse

Specific for human Feritin light chain denatured and native forms

Protein G Purifed

Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4

1.0 mg/ml (lot specific)

Store at -20 degree C

Small volumes of anti-FTL 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.

Stores iron in a soluble, non-toxic, readily available form. Important for iron homeostasis. Iron is taken up in the ferrous form and deposited as ferric hydroxides after oxidation. Also plays a role in delivery of iron to cells.Mediates iron uptake in capsule cells of the developing kidney.

Total protein Ab

Immunohistochemistry (IHC)

Immunohistochemistry: 1:20 - 1:200

Immunohistochemical analysis of paraffin-embedded human liver tissue using at dilution of 1:200.

Immunohistochemical analysis of paraffin-embedded human brain tissue using at dilution of 1:200.

Immunohistochemical analysis of paraffin-embedded human lymph node tissue using at dilution of 1:200.

20,020 Da[Similar Products]

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FRIL_HUMAN

FTL: Stores iron in a soluble, non-toxic, readily available form. Important for iron homeostasis. Iron is taken up in the ferrous form and deposited as ferric hydroxides after oxidation. Also plays a role in delivery of iron to cells. Mediates iron uptake in capsule cells of the developing kidney. Defects in FTL are the cause of hereditary hyperferritinemia-cataract syndrome (HHCS). It is an autosomal dominant disease characterized by early-onset bilateral cataract. Affected patients have elevated level of circulating ferritin. HHCS is caused by mutations in the iron responsive element (IRE) of the FTL gene. Defects in FTL are the cause of neurodegeneration with brain iron accumulation type 3 (NBIA3); also known as *****-onset basal ganglia disease. It is a movement disorder with heterogeneous presentations starting in the fourth to sixth decade. It is characterized by a variety of neurological signs including parkinsonism, ataxia, corticospinal signs, mild nonprogressive cognitive deficit and episodic psychosis. It is linked with decreased serum ferritin levels. Belongs to the ferritin family.

Protein type: Oxidoreductase

Chromosomal Location of Human Ortholog: 19q13.33

Cellular Component: cytoplasm; cytosol; ferritin complex; membrane

Molecular Function: identical protein binding; iron ion binding; protein binding

Biological Process: cellular iron ion homeostasis; intracellular sequestering of iron ion; iron ion homeostasis

Disease: Hyperferritinemia With Or Without Cataract; L-ferritin Deficiency; Neurodegeneration With Brain Iron Accumulation 3

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